Mast cell tumor differential diagnosis: Difference between revisions

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Revision as of 18:11, 25 January 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Overview

Mast cell tumor must be differentiated from other diseases that cause flushing,such as: phaeochromocytoma and carcinoid syndrome.^[1] Mast cell tumor must be differentiated from other diseases that cause elevated serum tryptase levels and cytopenia, such as: myelodysplastic syndrome, primary myelofibrosis, essential thrombocythemia, and chronic eosinophilic leukemia.

Differential Diagnosis

Mast cell tumor must be differentiated from other diseases that cause flushing:^[1]

Mast cell tumor must be differentiated from other diseases that cause abdominal pain and discomfort:

Mast cell tumor must be differentiated from other diseases that cause elevated serum tryptase levels and cytopenia, such as:

Differentiating Myeloproliferative Disorders

ABBREVIATIONS

N/A: Not available, NL: Normal, FISH: Fluorescence in situ hybridization, PCR: Polymerase chain reaction, LDH: Lactate dehydrogenase, PUD: Peptic ulcer disease, EPO: Erythropoietin, LFTs: Liver function tests, RFTs: Renal function tests, LAP: Leukocyte alkaline phosphatase, LAD: Leukocyte alkaline dehydrgenase, WBCs: White blood cells.

Myeloproliferative neoplasms (MPN)		Clinical manifestations		Diagnosis											Other features
		Symptoms	Physical examination	CBC & Peripheral smear									Bone marrow biopsy	Other investigations
				WBCs							Hb	Plat- elets
				Leuko-cytes	Blasts	Left shift	Baso- phils	Eosino- phils	Mono- cytes	Others	Hb	Plat- elets
Chronic myeloid leukemia (CML), BCR-ABL1+^[2]^[3]		Asymptomatic Constitutional Hyperviscosity and/or anemia related Bleeding Infection	Splenomegaly (46–76%) Purpura Anemia related Priapism	↑	<2%	+	↑	↑	↑	N/A	↓	NL	Hypercellurarity with ↑ granuloscytosis and ↓ erythrocytosis Fibrosis	FISH for t(9;22)(q34;q11.2) Reverse transcriptase quantitative PCR (RQ-PCR) for BCR-ABL	Granulocytic dysplasia is minimal/absent May present with blast crisis Absolute leukocytosis (median of 100,000/µL) Classic myelocyte bulge thrombocytopenia indicates advanced stage
Chronic neutrophilic leukemia (CNL)^[4]^[5]^[6]		Asymptomatic Constitutional symptoms Bleeding Infection	Splenomegaly Heptomegaly Purpura Anemia related	↑	Minimal	+	NL	NL	NL	↑ LDH ↑ B12 levels	↓	↓	Uniforme and intense hypercellularity with minimal to none fibrosis Neutrophil toxic granulations and Dohle bodies	FISH Imaging for hepatosplenomegaly	Associationed with polycythemia vera and plasma cell disorders Leukocytosis with chronic neutrophilia
Polycythemia vera (PV)^[7]^[8]^[9]^[10]		Constitutional Thromboembolism and bleeding Pruritus after a warm bath PUD related	Facial ruddiness Related to underlying cause Splenomegaly Renal bruit	NL or ↑	None	-	↑ or ↓	NL or ↑	NL	↓ Serum ferritin ↓ Folate levels ↑↑ B12 levels	↑↑	NL	Hypercellularity for age with tri-lineage growth Myelofibrosis (in up to 20% of patients)	Radioisotope studies Serum EPO levels LFTs RFTs Imaging studies	May transform into myelofibrosis or leukemia
Primary myelofibrosis (PMF)^[11]^[12]^[13]^[14]		Constitutional Anemia related Bleeding Infection Abdominal Pain	Hepatosplenomegaly Petechiae & ecchymoses Abdominal distension Lymphadenopathy	↓	Erythroblasts	-	Absent	NL	NL	↑ LAP ↑ LAD ↑ Uric acid ↑ B12 levels	↓	↓	Variable with fibrosis or hypercellularity	JAK2 mutation CALR mutation MPL mutation	Bone marrow aspiration shows a dry tap Variable with leukocytosis or leukopenia
Essential thrombocythemia (ET)^[15]^[16]^[17]		Headache Dizziness Visual disturbances Priapism Acute chest pain	Splenomegaly Skin bruises	NL or ↑	None	-	↓ or absent	NL	NL	N/A	↓	↑↑	Normal/Hypercellular	JAK2 mutation CALR mutation MPL mutation	Thrombosis Hemorrhage Pregnancy loss
Chronic eosinophilic leukemia, not otherwise specified (NOS)^[18]^[19]^[20]^[21]		Constitutional Rash Rhinitis Gastritis Thromboembolism related	Hypertension Eczema, mucosal ulcers, erythema Angioedema Ataxia Anemia Lymphadenopathy Hepatosplenomegaly	↑	Present	+	↑	↑↑	↑	↑ B12 levels ↑ LDH	↓	↓	Hypercelluar with ↑ eosinophilic precursors, ↑ eosinophils, and atypical mononuclear cells	FISH Cytogenetic analysis of purified eosinophils and X-chromosome inactivation analysis	Heart failure Lung fibrosis Encephalopathy Erythema annulare centrifugam
MPN, unclassifiable		Similar to other myeloproliferative neoplasms	Similar to other myeloproliferative neoplasms	↑	Variable	±	↑ or ↓	↑ or ↓	↑ or ↓	May resemble other myeloproliferative neoplasms	↓	↑	↑ megakaryocyte proliferation with variable hypercellularity in granulocytic or erythrocytic cell lines	N/A	Similar to other myeloprolifeartive neoplasms but do not fulfil the criteria to be classified to a specific type
Mastocytosis^[22]^[23]^[24]^[25]		Constitutional Pruritus & Flushing Urticaria & Blisters Hypotension & PUD Bleeding Bronchoconstriction	Mastocytosis exanthema Blistering Swelling Lymphadenopathy Bleeding Fibrosis	↑	None	-	NL	↑	NL	↑ Alkaline phosphatase ↑ LDH	↓	↓ or ↑	Multifocal dense infiltrates of mast cells with atypical morphology in >25 %	Cytogenetic analysis for c-KIT receptor mutations Serum tryptase levels 24-hour urine test for N-methyl histamine and 11-beta-prostaglandine	Skin most commonly involved Susceptibility to anaphylaxix Osteoporosis
Myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, or FGFR1, or with PCM1-JAK2^[26]^[27]^[28]^[29]		Asymptomatic Constitutional Rash Cough & breathlessness Peripheral neuropathy/ encephalopathy	Fever Lymphadenopathy	↑	NL	-	NL	↑	↑	None	NL	↓	Myeloid expansion with eosinophilia	FISH shows t(8;13) and t(8;22)	May present or evolve into acute myeloid or lymphoblastic leukemia Leukocytosis (30 - 59 × 10⁹/L
B-lymphoblastic leukemia/lymphoma^[30]^[31]		Constitutional Anemia related Bleeding Infection Bone pain	Pallor Petechiae Organomegaly Lymphadenopathy	NL or ↑	>25%	N/A	↑ or ↓	↑ or ↓	↑ or ↓	Auer bodies	↓	↓	Hypercellular with blast infilterationwith or without myelodysplasia	Cytogenetic analysis Flow cytometry FISH	May present as extramedullary disease (Myeloid sarcoma)
Myelodysplastic syndromes (MDS)^[32]^[33]		Constitutional Anemia related Bleeding Infection	Pallor Petechiae Organomegaly	↓	Variable	-	↓	↓	↓	Macro-ovalocytes Basophilic stippling Howell-Jolly body	↓	↓	Hypercellular/ normocellular bone marrow with dysplastic changes	Cytogenetic analysis Flow cytometry	Leukemia transformation Acquired pseudo-Pelger-Huët anomaly
Acute myeloid leukemia (AML) and related neoplasms^[34]^[35]		Constitutional Anemia related Bleeding Bone pain Joint pain Infections	Infection related Pallor Leukemia cutis Bruising & petechiae Lymphadenopathy Hepatosplenomegaly	NL or ↑	↑	N/A	↑ or ↓	↑ or ↓	↑ or ↓	↑ Potassium ↑ Uric acid ↑ Phosphorus ↓ Calcium ↑ LDH	↓	↓	Increased immaturemyeloid cells with dysplasia	Cytogenetic analysis Flow cytometry FISH	Common in Down syndrome
Blastic plasmacytoid dendritic cell neoplasm^[36]^[37]^[38]^[39]		Cutaneous symptoms (brown/purple nodular lesions) on face, scalp, lower limb & trunk	Brown/violaceous bruise like lesions Lymphadenopathy Splenomegaly	NL	↑		NL	NL	NL	Neutropenia	↓	↓	Malignant cells	Immunohistochemistry or flow cytometry for CD4 & CD56	TdT expression positive May develop chronic myelomonocytic leukemia (CMML)
Myelodysplastic /myeloproliferative neoplasms (MDS/MPN)	Chronic myelomonocytic leukemia (CMML)^[40] ^[41]^[42]	Constitutional Anemia related Bleeding Infections Bone pain Leukemia Cutis	Organomegaly Bruising	↑	< 20%		NL	↑	↑↑	↑ LDH	↓	↓	Myelodysplastic and myeloproliferative feature	Cytogenetic analysis Flow cytometry	Overlapping of both, MDS and MPN Absolute monocytosis > 1 × 10⁹/L (defining feature) MD-CMML:WBC ≤ 13 × 10⁹/L (FAB) MP-CMML:WBC > 13 × 10⁹/L (FAB)
	Atypical chronic myeloid leukemia (aCML), BCR-ABL1-^[43]^[44]	Asymptomatic Constitutional Hyperviscosity and/or anemia related Bleeding Infection	Splenomegaly (46–76%) Purpura Anemia related Priapism	↑	<20%	+	<2% of WBCs	N/A	N/A	N/A	↓	↓	Granulocytic hyperplasia with prominent dysplasia	Cytogenetic analysis Flow cytometry	Granulocytic dysplasia is prominent Absence of BCR-ABL or PDGFRA, PDGFRB, or FGFR1 rearrangements WBC > 13 × 10⁹/L
	Juvenile myelomonocytic leukemia (JMML)^[45]^[46]	Infections Anemia related	Hepatosplenomegaly Lymphadenopathy Rash	↑	↑	N/A	N/A	N/A	↑	↓ Serum Iron ↑ B12 levels	↓	↓	Hypercelluar with ↑ myeloid cells in stages of maturation	Cytogenetic analysis Flow cytometry	Polyclonal hypergammaglobulinemia
	MDS/MPN with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T)^[47]^[48]^[49]	Constitutional Anemia related Thrombosis	Variable	NL or ↑	NL	-	NL	N/A	N/A	↑ Serum Iron	↓	↑	Hypercellularity with dyserythropoiesis and increased megakaryocytes	Cytogenetic analysis Flow cytometry	Large atypical megakaryocytes Ringed sideroblasts SF3B1 mutation
T-lymphoblastic leukemia/ lymphoma	T-lymphoblastic leukemia/ lymphoma^[50]^[51]^[52]	Constitutional Anemia Related Bleeding Superior vena cava syndrome	Lymphadenopathy Mediastinal mass Pleural effusions Tracheal obstruction Pericardial effusions	↑	>25% blasts (Leukemia) <25% blasts (Lymphoma)	±	↑ or ↓	↑ or ↓	↑ or ↓	↑ LDH Positive for TdT	↓	↓	Hypercelluarity with increased T cells precursors	Cytogenetic analysisFlow cytometry FISH	May involve brain, skin, and testes.
	Provisional entity: Natural killer (NK) cell lymphoblastic leukemia/lymph^[53]	Constitutional Anemia Related Bleeding Superior vena cava syndrome	Lymphadenopathy Mediastinal mass Pleural effusions Tracheal obstruction Pericardial effusions	↑	↑	±	↑ or ↓	↑ or ↓	↑ or ↓	↑ LDH	↓	↓	N/A	Cytogenetic analysis FISH Flow cytometry	Similar to T-cell lymphoblastic leukemia but may have more aggressive clinical course. Diagnosis is usually based on presence of CD56 expression, and T-cell-associated markers such as CD2 and CD7. B-cell markers are absent.
	Provisional entity: Early T-cell precursor lymphoblastic leukemia^[54]^[55]	Constitutional Anemia Related Bleeding Superior vena cava syndrome	Lymphadenopathy Mediastinal mass Pleural effusions Tracheal obstruction Pericardial effusions	↑	↑	±	↑ or ↓	↑ or ↓	↑ or ↓	↑ LDH	↓	↓	Hypercelluarity with increased T cells precursors	Cytogenetic analysis FISH Flow cytometry	Similar to T-cell lymphoblastic leukemia but is more aggressive clinically and cell are characterized by cytometry as CD1a⁻, CD8⁻, CD5⁻ (dim), and positivity for 1 or more stem cell or myeloid antigens. Gene expression indicates more immature cells as compared to other subtypes of T-cell neoplasms.

References

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↑ You MJ, Medeiros LJ, Hsi ED (September 2015). "T-lymphoblastic leukemia/lymphoma". Am. J. Clin. Pathol. 144 (3): 411–22. doi:10.1309/AJCPMF03LVSBLHPJ. PMID 26276771.
↑ Patel KJ, Latif SU, de Calaca WM (March 2009). "An unusual presentation of precursor T cell lymphoblastic leukemia/lymphoma with cholestatic jaundice: case report". J Hematol Oncol. 2: 12. doi:10.1186/1756-8722-2-12. PMC 2663564. PMID 19284608.
↑ Elreda L, Sandhu M, Sun X, Bekele W, Cohen AJ, Shah M (2014). "T-cell lymphoblastic leukemia/lymphoma: relapse 16 years after first remission". Case Rep Hematol. 2014: 359158. doi:10.1155/2014/359158. PMC 4005062. PMID 24822133.
↑ Sedick Q, Alotaibi S, Alshieban S, Naheet KB, Elyamany G (2017). "Natural Killer Cell Lymphoblastic Leukaemia/Lymphoma: Case Report and Review of the Recent Literature". Case Rep Oncol. 10 (2): 588–595. doi:10.1159/000477843. PMID 28868017.
↑ Jain N, Lamb AV, O'Brien S, Ravandi F, Konopleva M, Jabbour E, Zuo Z, Jorgensen J, Lin P, Pierce S, Thomas D, Rytting M, Borthakur G, Kadia T, Cortes J, Kantarjian HM, Khoury JD (April 2016). "Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) in adolescents and adults: a high-risk subtype". Blood. 127 (15): 1863–9. doi:10.1182/blood-2015-08-661702. PMC 4915808. PMID 26747249.
↑ Haydu JE, Ferrando AA (July 2013). "Early T-cell precursor acute lymphoblastic leukaemia". Curr. Opin. Hematol. 20 (4): 369–73. doi:10.1097/MOH.0b013e3283623c61. PMC 3886681. PMID 23695450.

Template:WH Template:WS

[Patient-1] 1.0 ^1.1 Mastocytosis and mast cell disorders.Patient info.http://patient.info/doctor/mastocytosis-and-mast-cell-disorders#ref-20 accessed on March 7th, 2016

[pmid9012696-2] Savage DG, Szydlo RM, Goldman JM (January 1997). "Clinical features at diagnosis in 430 patients with chronic myeloid leukaemia seen at a referral centre over a 16-year period". Br. J. Haematol. 96 (1): 111–6. PMID 9012696.

[pmid26434969-3] Thompson PA, Kantarjian HM, Cortes JE (October 2015). "Diagnosis and Treatment of Chronic Myeloid Leukemia in 2015". Mayo Clin. Proc. 90 (10): 1440–54. doi:10.1016/j.mayocp.2015.08.010. PMC 5656269. PMID 26434969.

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@@ Line 21: / Line 21: @@
 *Myelomastocytic leukemia
 *[[Malignant lymphoma]]
+==Differentiating Myeloproliferative Disorders==
+'''<small>ABBREVIATIONS'''
+'''N/A''': Not available, '''NL''': Normal, '''FISH''': Fluorescence in situ hybridization, '''PCR''': Polymerase chain reaction, '''LDH''': Lactate dehydrogenase, '''PUD''': Peptic ulcer disease, '''EPO''': Erythropoietin, '''LFTs''': Liver function tests, '''RFTs''': Renal function tests, '''LAP''': Leukocyte alkaline phosphatase, '''LAD''': Leukocyte alkaline dehydrgenase, '''WBCs''': White blood cells. </small>
+<small>
+{| class="wikitable"
+! colspan="2" rowspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" + |Myeloproliferative neoplasms (MPN)
+! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Clinical manifestations
+! colspan="11" style="background:#4479BA; color: #FFFFFF;" align="center" + |Diagnosis
+! rowspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" + |Other features
+|-
+! rowspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Symptoms
+! rowspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Physical examination
+! colspan="9" style="background:#4479BA; color: #FFFFFF;" align="center" + |CBC & Peripheral smear
+! rowspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Bone marrow biopsy
+! rowspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Other investigations
+|-
+! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" + |WBCs
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Hb
+! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Plat-<br>elets
+|-
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Leuko-cytes</small>
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Blasts</small>
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Left<br>shift</small>
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Baso-<br>phils</small>
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Eosino-<br>phils</small>
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Mono-<br>cytes</small>
+! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Others</small>
+|-
+| colspan="2" |[[Chronic myeloid leukemia]]<br>([[CML]]), [[BCR/ABL|BCR-ABL1]]+<ref name="pmid9012696">{{cite journal |vauthors=Savage DG, Szydlo RM, Goldman JM |title=Clinical features at diagnosis in 430 patients with chronic myeloid leukaemia seen at a referral centre over a 16-year period |journal=Br. J. Haematol. |volume=96 |issue=1 |pages=111–6 |date=January 1997 |pmid=9012696 |doi= |url=}}</ref><ref name="pmid26434969">{{cite journal |vauthors=Thompson PA, Kantarjian HM, Cortes JE |title=Diagnosis and Treatment of Chronic Myeloid Leukemia in 2015 |journal=Mayo Clin. Proc. |volume=90 |issue=10 |pages=1440–54 |date=October 2015 |pmid=26434969 |pmc=5656269 |doi=10.1016/j.mayocp.2015.08.010 |url=}}</ref>
+|
+* <small>Asymptomatic</small>
+* <small>Constitutional</small>
+* <small>[[Hyperviscosity]]  and/or [[anemia]] related</small>
+* <small>[[Bleeding]]</small>
+* <small>[[Infection]]</small>
+|
+* <small>[[Splenomegaly]]<br>(46–76%)</small>
+* <small>[[Purpura]]</small>
+* <small>[[Anemia]] related</small>
+* <small>[[Priapism]]</small>
+|↑
+|<small><2%</small>
+| +
+|<small>↑</small>
+|<small>↑</small>
+|<small>↑</small>
+|<small>N/A</small>
+|<small>[[Anemia|↓]]</small>
+|<small>NL</small>
+|
+* <small>Hypercellurarity with ↑ [[granuloscytosis]] and ↓ [[erythrocytosis]]</small>
+* <small>[[Fibrosis]]</small>
+|
+* <small>[[FISH]] for t(9;22)(q34;q11.2)</small>
+* <small>[[Reverse transcriptase]] quantitative [[PCR]] (RQ-PCR) for BCR-ABL</small>
+|
+* <small>[[Granulocytic]] [[dysplasia]] is minimal/absent</small>
+* <small>May present with [[blast]] crisis</small>
+* <small>Absolute [[leukocytosis]]</small>  <small>(median of 100,000/µL)</small>
+* <small>Classic [[myelocyte]] bulge</small>
+* <small>[[thrombocytopenia]] indicates advanced stage</small>
+|-
+| colspan="2" |[[Chronic neutrophilic leukemia]] ([[CNL]])<ref name="pmid29440636">{{cite journal |vauthors=Szuber N, Tefferi A |title=Chronic neutrophilic leukemia: new science and new diagnostic criteria |journal=Blood Cancer J |volume=8 |issue=2 |pages=19 |date=February 2018 |pmid=29440636 |pmc=5811432 |doi=10.1038/s41408-018-0049-8 |url=}}</ref><ref name="pmid28028025">{{cite journal |vauthors=Maxson JE, Tyner JW |title=Genomics of chronic neutrophilic leukemia |journal=Blood |volume=129 |issue=6 |pages=715–722 |date=February 2017 |pmid=28028025 |pmc=5301820 |doi=10.1182/blood-2016-10-695981 |url=}}</ref><ref name="pmid26366092">{{cite journal |vauthors=Menezes J, Cigudosa JC |title=Chronic neutrophilic leukemia: a clinical perspective |journal=Onco Targets Ther |volume=8 |issue= |pages=2383–90 |date=2015 |pmid=26366092 |pmc=4562747 |doi=10.2147/OTT.S49688 |url=}}</ref>
+|
+* <small>Asymptomatic</small>
+* <small>Constitutional symptoms</small>
+* <small>[[Bleeding]]</small>
+* <small>[[Infection]]</small>
+|
+* <small>[[Splenomegaly]]</small>
+* <small>[[Heptomegaly]]</small>
+* <small>[[Purpura]]</small>
+* <small>[[Anemia]] related</small>
+|↑
+|<small>Minimal</small>
+| +
+|<small>NL</small>
+|<small>NL</small>
+|<small>NL</small>
+|
+* <small>↑  [[LDH]]</small>
+* <small>↑ [[B12]] levels</small>
+|<small>[[Anemia|↓]]</small>
+|<small>[[Thrombocytopenia|↓]]</small>
+|
+* <small>Uniforme and intense hypercellularity with minimal to none [[fibrosis]]</small>
+* <small>[[Neutrophil]] toxic granulations and [[Dohle bodies]]</small>
+|
+* <small>[[FISH]]</small>
+* <small>Imaging for [[hepatosplenomegaly]]</small>
+|
+* <small>Associationed with [[polycythemia vera]] and [[plasma cell disorders]]</small>
+* <small>[[Leukocytosis]] with<br>[[chronic]] [[neutrophilia]]</small>
+|-
+| colspan="2" |[[Polycythemia vera]]<br>([[PV]])<ref name="pmid29194068">{{cite journal |vauthors=Vannucchi AM, Guglielmelli P, Tefferi A |title=Polycythemia vera and essential thrombocythemia: algorithmic approach |journal=Curr. Opin. Hematol. |volume=25 |issue=2 |pages=112–119 |date=March 2018 |pmid=29194068 |doi=10.1097/MOH.0000000000000402 |url=}}</ref><ref name="pmid30252337">{{cite journal |vauthors=Pillai AA, Babiker HM |title= |journal= |volume= |issue= |pages= |date= |pmid=30252337 |doi= |url=}}</ref><ref name="pmid30281843">{{cite journal |vauthors=Tefferi A, Barbui T |title=Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management |journal=Am. J. Hematol. |volume=94 |issue=1 |pages=133–143 |date=January 2019 |pmid=30281843 |doi=10.1002/ajh.25303 |url=}}</ref><ref name="pmid28028026">{{cite journal |vauthors=Rumi E, Cazzola M |title=Diagnosis, risk stratification, and response evaluation in classical myeloproliferative neoplasms |journal=Blood |volume=129 |issue=6 |pages=680–692 |date=February 2017 |pmid=28028026 |pmc=5335805 |doi=10.1182/blood-2016-10-695957 |url=}}</ref>
+|
+* <small>Constitutional</small>
+*  <small>[[Thromboembolism]]<br>and [[bleeding]]</small>
+* <small>[[Pruritus]] after<br>a warm bath</small>
+* <small>[[PUD]] related</small>
+|
+* <small>Facial ruddiness</small>
+* <small>Related to underlying cause</small>
+* <small>[[Splenomegaly]]</small>
+* <small>[[Renal]] [[bruit]]</small>
+|<small>NL or ↑</small>
+|<small>None</small>
+| -
+|<small>↑ or ↓</small>
+|<small>NL or ↑</small>
+|<small>NL</small>
+|
+* <small>↓ Serum [[ferritin]]</small>
+* <small>↓ [[Folate]] levels</small>
+* <small>↑↑ [[B12]] levels</small>
+|<small>↑↑</small>
+|<small>NL</small>
+|
+* <small>Hypercellularity for age with tri-lineage growth</small>
+* <small>[[Myelofibrosis]] (in up to 20% of patients)</small>
+|
+* <small>[[Radioisotope]] studies</small>
+* <small>[[Serum]] [[EPO]] levels</small>
+* <small>[[LFTs]]</small>
+* <small>[[RFTs]]</small>
+* <small>[[Imaging]] studies</small>
+|
+* <small>May transform into [[myelofibrosis]] or [[leukemia]]</small>
+|-
+| colspan="2" |[[Primary myelofibrosis]] ([[PMF]])<ref name="pmid26891375">{{cite journal |vauthors=Cervantes F, Correa JG, Hernandez-Boluda JC |title=Alleviating anemia and thrombocytopenia in myelofibrosis patients |journal=Expert Rev Hematol |volume=9 |issue=5 |pages=489–96 |date=May 2016 |pmid=26891375 |doi=10.1586/17474086.2016.1154452 |url=}}</ref><ref>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref><ref name="pmid16919893">{{cite journal |vauthors=Michiels JJ, Bernema Z, Van Bockstaele D, De Raeve H, Schroyens W |title=Current diagnostic criteria for the chronic myeloproliferative disorders (MPD) essential thrombocythemia (ET), polycythemia vera (PV) and chronic idiopathic myelofibrosis (CIMF) |journal=Pathol. Biol. |volume=55 |issue=2 |pages=92–104 |date=March 2007 |pmid=16919893 |doi=10.1016/j.patbio.2006.06.002 |url=}}</ref><ref>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref>
+|
+* <small>Constitutional</small>
+* <small>[[Anemia]] related</small>
+* <small>[[Bleeding]]</small>
+* <small>[[Infection]]</small>
+* <small>[[Abdominal]] [[Pain]]</small>
+|
+* <small>[[Hepatosplenomegaly]]</small>
+* <small>[[Petechiae]] & [[ecchymoses]]</small>
+* <small>Abdominal distension</small>
+* <small>[[Lymphadenopathy]]</small>
+|↓
+|<small>[[Erythroblasts]]</small>
+| -
+|<small>Absent</small>
+|<small>NL</small>
+|<small>NL</small>
+|
+* <small>↑ [[LAP]]</small>
+* <small>↑ [[LAD]]</small>
+* ↑ <small>[[Uric acid]]</small>
+* <small>↑ [[B12]] levels</small>
+|[[Anemia|<small>↓</small>]]
+|<small>[[Thrombocytopenia|↓]]</small>
+|
+* <small>Variable with [[fibrosis]] or hypercellularity</small>
+|
+* <small>JAK2 mutation</small>
+* <small>[[CALR]] [[mutation]]</small>
+* <small>[[MPL]] [[mutation]]</small>
+|
+* <small>[[Bone marrow]] aspiration shows a dry tap</small>
+* <small>Variable with [[leukocytosis]] or [[leukopenia]]</small>
+|-
+| colspan="2" |[[Essential thrombocythemia]] ([[ET]])<ref name="pmidhttp://dx.doi.org/10.1182/blood-2007-04-083501">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=http://dx.doi.org/10.1182/blood-2007-04-083501 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10  }} </ref><ref>{{Cite journal
+ | author = [[Daniel A. Arber]], [[Attilio Orazi]], [[Robert Hasserjian]], [[Jurgen Thiele]], [[Michael J. Borowitz]], [[Michelle M. Le Beau]], [[Clara D. Bloomfield]], [[Mario Cazzola]] & [[James W. Vardiman]]
+ | title = The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia
+ | journal = [[Blood]]
+ | volume = 127
+ | issue = 20
+ | pages = 2391–2405
+ | year = 2016
+ | month = May
+ | doi = 10.1182/blood-2016-03-643544
+ | pmid = 27069254
+}}</ref><ref>{{Cite journal
+ | author = [[A. Tefferi]], [[R. Fonseca]], [[D. L. Pereira]] & [[H. C. Hoagland]]
+ | title = A long-term retrospective study of young women with essential thrombocythemia
+ | journal = [[Mayo Clinic proceedings]]
+ | volume = 76
+ | issue = 1
+ | pages = 22–28
+ | year = 2001
+ | month = January
+ | doi = 10.4065/76.1.22
+ | pmid = 11155408
+}}</ref>
+|
+* <small>Headache</small>
+* <small>[[Dizziness]]</small>
+* <small>[[Visual]] disturbances</small>
+* <small>[[Priapism]]</small>
+* <small>Acute [[chest pain]]</small>
+|
+* <small>[[Splenomegaly]]</small>
+* <small>[[Skin]] [[bruises]]</small>
+|
+<small>NL or ↑</small>
+|
+<small>None</small>
+| -
+|
+<small>↓ or absent</small>
+|
+<small>NL</small>
+|
+<small>NL</small>
+|
+* <small>N/A</small>
+|
+[[Anemia|<small>↓</small>]]
+|
+<small>↑↑</small>
+|
+* <small>Normal/Hypercellular</small>
+|
+* <small>[[JAK2]] [[mutation]]</small>
+* <small>[[CALR]] [[mutation]]</small>
+* <small>[[MPL]] [[mutation]]</small>
+|
+* <small>[[Thrombosis]]</small>
+* <small>[[Hemorrhage]]</small>
+* <small>[[Pregnancy]] loss</small>
+|-
+| colspan="2" |[[Chronic eosinophilic leukemia]],<br>not otherwise specified<br>(NOS)<ref name="pmid27512192">{{cite journal |vauthors=Vidyadharan S, Joseph B, Nair SP |title=Chronic Eosinophilic Leukemia Presenting Predominantly with Cutaneous Manifestations |journal=Indian J Dermatol |volume=61 |issue=4 |pages=437–9 |date=2016 |pmid=27512192 |pmc=4966405 |doi=10.4103/0019-5154.185716 |url=}}</ref><ref name="pmid29892549">{{cite journal |vauthors=Hofmans M, Delie A, Vandepoele K, Van Roy N, Van der Meulen J, Philippé J, Moors I |title=A case of chronic eosinophilic leukemia with secondary transformation to acute myeloid leukemia |journal=Leuk Res Rep |volume=9 |issue= |pages=45–47 |date=2018 |pmid=29892549 |pmc=5993353 |doi=10.1016/j.lrr.2018.04.001 |url=}}</ref><ref name="pmid23662039">{{cite journal |vauthors=Yamada Y, Rothenberg ME, Cancelas JA |title=Current concepts on the pathogenesis of the hypereosinophilic syndrome/chronic eosinophilic leukemia |journal=Transl Oncogenomics |volume=1 |issue= |pages=53–63 |date=2006 |pmid=23662039 |pmc=3642145 |doi= |url=}}</ref><ref name="pmid27722133">{{cite journal |vauthors=Kim TH, Gu HJ, Lee WI, Lee J, Yoon HJ, Park TS |title=Chronic eosinophilic leukemia with FIP1L1-PDGFRA rearrangement |journal=Blood Res |volume=51 |issue=3 |pages=204–206 |date=September 2016 |pmid=27722133 |doi=10.5045/br.2016.51.3.204 |url=}}</ref>
+|
+* <small>Constitutional</small>
+* <small>[[Rash]]</small>
+* <small>[[Rhinitis]]</small>
+* <small>[[Gastritis]]</small>
+* <small>[[Thromboembolism]]<br>related</small>
+|
+* <small>[[Hypertension]]</small>
+* <small>[[Eczema]], [[mucosal]] [[ulcers]], [[erythema]]</small>
+* <small>[[Angioedema]]</small>
+* <small>[[Ataxia]]</small>
+* <small>[[Anemia]]</small>
+* <small>[[Lymphadenopathy]]</small>
+* <small>[[Hepatosplenomegaly]]</small>
+|<small>[[Leukocytosis|↑]]</small>
+|<small>Present</small>
+| +
+|<small>↑</small>
+|<small>↑↑</small>
+|<small>↑</small>
+|
+* <small>↑ [[B12]] levels</small>
+* ↑ <small>[[LDH]]</small>
+|<small>[[Anemia|↓]]</small>
+|<small>[[Thrombocytopenia|↓]]</small>
+|
+* <small>Hypercelluar with  ↑ [[eosinophilic]] precursors, ↑ [[eosinophils]], and atypical [[mononuclear cells]]</small>
+|
+* <small>[[FISH]]</small>
+* <small>Cytogenetic analysis of purified [[eosinophils]] and [[X-chromosome]] inactivation analysis</small>
+|
+* <small>[[Heart failure]]</small> <small>[[Lung fibrosis]]</small>
+* <small>[[Encephalopathy]]</small>
+* <small>[[Erythema annulare centrifugam]]</small>
+|-
+| colspan="2" |[[Myeloproliferative neoplasm|MPN]],<br>unclassifiable
+|<small>
+*Similar to other<br>[[Myeloproliferative neoplasm|myeloproliferative neoplasms]]</small>
+|<small>
+*Similar to other<br>[[Myeloproliferative neoplasm|myeloproliferative neoplasms]]</small>
+|<small>[[Leukocytosis|↑]]</small>
+|<small>Variable</small>
+|±
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|
+* <small>May resemble other<br>[[myeloproliferative neoplasms]]</small>
+|<small>↓</small>
+|<small>↑</small>
+|
+* <small>↑ [[megakaryocyte]] proliferation with variable hypercellularity in [[granulocytic]] or [[erythrocytic]] cell lines</small>
+|
+* <small>N/A</small>
+|<small>
+*Similar to other [[myeloprolifeartive neoplasms]] but do not fulfil the criteria to be classified to a specific type</small>
+|-
+| colspan="2" |[[Mastocytosis]]<ref name="pmid24262698">{{cite journal |vauthors=Carter MC, Metcalfe DD, Komarow HD |title=Mastocytosis |journal=Immunol Allergy Clin North Am |volume=34 |issue=1 |pages=181–96 |date=February 2014 |pmid=24262698 |pmc=3863935 |doi=10.1016/j.iac.2013.09.001 |url=}}</ref><ref name="pmid29494109">{{cite journal |vauthors=Macri A, Cook C |title= |journal= |volume= |issue= |pages= |date= |pmid=29494109 |doi= |url=}}</ref><ref name="pmid25031064">{{cite journal |vauthors=Lladó AC, Mihon CE, Silva M, Galzerano A |title=Systemic mastocytosis - a diagnostic challenge |journal=Rev Bras Hematol Hemoter |volume=36 |issue=3 |pages=226–9 |date=2014 |pmid=25031064 |pmc=4109736 |doi=10.1016/j.bjhh.2014.03.003 |url=}}</ref><ref name="pmid28031180">{{cite journal |vauthors=Valent P, Akin C, Metcalfe DD |title=Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts |journal=Blood |volume=129 |issue=11 |pages=1420–1427 |date=March 2017 |pmid=28031180 |pmc=5356454 |doi=10.1182/blood-2016-09-731893 |url=}}</ref>
+|
+* <small>Constitutional</small>
+* <small>[[Pruritus]] & [[Flushing]]</small>
+* <small>[[Urticaria]] & [[Blisters]]</small>
+* <small>[[Hypotension]] & [[PUD]]</small>
+* <small>[[Bleeding]]</small>
+* <small>[[Bronchoconstriction]]</small>
+|
+* <small>[[Mastocytosis exanthema]]</small>
+* <small>[[Blistering]]</small>
+* <small>[[Swelling]]</small>
+* <small>[[Lymphadenopathy]]</small>
+* <small>[[Bleeding]]</small>
+* <small>[[Fibrosis]]</small>
+|<small>[[Leukocytosis|↑]]</small>
+|<small>None</small>
+| -
+|<small>NL</small>
+|<small>[[Eosinophilia|↑]]</small>
+|<small>NL</small>
+|
+* <small>↑ [[Alkaline phosphatase]]</small>
+* <small>↑ [[LDH]]</small>
+|<small>[[Anemia|↓]]</small>
+|<small>↓ or ↑</small>
+|
+* <small>Multifocal dense infiltrates of [[mast cells]] with atypical [[morphology]] in >25 %</small>
+|
+* <small>Cytogenetic analysis for [[c-KIT]] [[receptor]] [[mutations]]</small>
+* <small>[[Serum]] [[tryptase]] levels</small>
+* <small>24-hour urine test for N-methyl [[histamine]] and 11-beta-[[prostaglandine]]</small>
+|
+* <small>Skin most commonly involved</small>
+* <small>Susceptibility to [[anaphylaxix]]</small>
+* <small>[[Osteoporosis]]</small>
+|-
+| colspan="2" |[[Myeloid]]/[[lymphoid]] [[neoplasms]]<br>with [[eosinophilia]] and rearrangement<br>of [[PDGFR|PDGFRA]], [[PDGFRB]], or [[FGFR1]],<br>or with [[PCM1]]-[[JAK2]]<ref name="KumarChen2015">{{cite journal|last1=Kumar|first1=Kirthi R.|last2=Chen|first2=Weina|last3=Koduru|first3=Prasad R.|last4=Luu|first4=Hung S.|title=Myeloid and Lymphoid Neoplasm With Abnormalities of FGFR1 Presenting With Trilineage Blasts and RUNX1 Rearrangement|journal=American Journal of Clinical Pathology|volume=143|issue=5|year=2015|pages=738–748|issn=1943-7722|doi=10.1309/AJCPUD6W1JLQQMNA}}</ref><ref>{{Cite journal
+ | author = [[Paolo Strati]], [[Guilin Tang]], [[Dzifa Y. Duose]], [[Saradhi Mallampati]], [[Rajyalakshmi Luthra]], [[Keyur P. Patel]], [[Mohammad Hussaini]], [[Abu-Sayeef Mirza]], [[Rami S. Komrokji]], [[Stephen Oh]], [[John Mascarenhas]], [[Vesna Najfeld]], [[Vivek Subbiah]], [[Hagop Kantarjian]], [[Guillermo Garcia-Manero]], [[Srdan Verstovsek]] & [[Naval Daver]]
+ | title = Myeloid/lymphoid neoplasms with FGFR1 rearrangement
+ | journal = [[Leukemia & lymphoma]]
+ | volume = 59
+ | issue = 7
+ | pages = 1672–1676
+ | year = 2018
+ | month = July
+ | doi = 10.1080/10428194.2017.1397663
+ | pmid = 29119847
+}}</ref><ref>{{Cite journal
+ | author = [[Ximena Montenegro-Garreaud]], [[Roberto N. Miranda]], [[Alexandra Reynolds]], [[Guilin Tang]], [[Sa A. Wang]], [[Mariko Yabe]], [[Wei Wang]], [[Lianghua Fang]], [[Carlos E. Bueso-Ramos]], [[Pei Lin]], [[L. Jeffrey Medeiros]] & [[Xinyan Lu]]
+ | title = Myeloproliferative neoplasms with t(8;22)(p11.2;q11.2)/BCR-FGFR1: a meta-analysis of 20 cases shows cytogenetic progression with B-lymphoid blast phase
+ | journal = [[Human pathology]]
+ | volume = 65
+ | pages = 147–156
+ | year = 2017
+ | month = July
+ | doi = 10.1016/j.humpath.2017.05.008
+ | pmid = 28551329
+}}</ref><ref>{{Cite journal
+ | author = [[Paola Villafuerte-Gutierrez]], [[Montserrat Lopez Rubio]], [[Pilar Herrera]] & [[Eva Arranz]]
+ | title = A Case of Myeloproliferative Neoplasm with BCR-FGFR1 Rearrangement: Favorable Outcome after Haploidentical Allogeneic Transplantation
+ | journal = [[Case reports in hematology]]
+ | volume = 2018
+ | pages = 5724960
+ | year = 2018
+ | month =
+ | doi = 10.1155/2018/5724960
+ | pmid = 30647980
+}}</ref>
+|
+* <small>Asymptomatic</small>
+* <small>Constitutional</small>
+* <small>[[Rash]]</small>
+* <small>[[Cough]] & breathlessness</small>
+* <small>[[Peripheral neuropathy]]/<br>[[encephalopathy]]</small>
+|
+* <small>[[Fever]]</small>
+* <small>[[Lymphadenopathy]]</small>
+| ↑
+| <small>NL</small>
+| -
+| <small>NL</small>
+| <small>[[Eosinophilia|↑]]</small>
+|<small>[[Monocytosis|↑]]</small>
+|
+* <small>None</small>
+| <small>NL</small>
+| <small>[[Thrombocytopenia|↓]]</small>
+|
+* <small>[[Myeloid]] expansion with [[eosinophilia]]</small>
+|
+* <small>[[FISH]] shows t(8;13) and t(8;22)</small>
+|
+* <small>May present or evolve into [[acute myeloid]] or [[lymphoblastic leukemia]]</small>
+* <small>[[Leukocytosis]] (30 - 59 × 10<sup>9</sup>/L</small>
+|-
+| colspan="2" |B-lymphoblastic leukemia/lymphoma<ref name="pmid30107017">{{cite journal |vauthors=Kamiya-Matsuoka C, Garciarena P, Amin HM, Tremont-Lukats IW, de Groot JF |title=B lymphoblastic leukemia/lymphoma presenting as seventh cranial nerve palsy |journal=Neurol Clin Pract |volume=3 |issue=6 |pages=532–534 |date=December 2013 |pmid=30107017 |pmc=6082360 |doi=10.1212/CPJ.0b013e3182a78ef0 |url=}}</ref><ref name="pmid29029550">{{cite journal |vauthors=Zhang X, Rastogi P, Shah B, Zhang L |title=B lymphoblastic leukemia/lymphoma: new insights into genetics, molecular aberrations, subclassification and targeted therapy |journal=Oncotarget |volume=8 |issue=39 |pages=66728–66741 |date=September 2017 |pmid=29029550 |pmc=5630450 |doi=10.18632/oncotarget.19271 |url=}}</ref>
+|
+* <small>Constitutional</small>
+* <small>[[Anemia]] related</small>
+* <small>[[Bleeding]]</small>
+* <small>[[Infection]]</small>
+* <small>[[Bone pain]]</small>
+|
+* <small>[[Pallor]]</small>
+* <small>[[Petechiae]]</small>
+* <small>[[Organomegaly]]</small>
+* <small>[[Lymphadenopathy]]</small>
+|<small>NL or ↑</small>
+|<small>>25%</small>
+|<small>N/A</small>
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|
+* <small>[[Auer rod|Auer bodies]]</small>
+|[[Anemia|<small>↓</small>]]
+|<small>[[Thrombocytopenia|↓]]</small>
+|
+* <small>Hypercellular with [[blast]] infilteration</small><small>with or without [[myelodysplasia]]</small>
+|
+* <small>[[Cytogenetic analysis]]</small>
+* <small>[[Flow cytometry]]</small>
+* <small>[[FISH]]</small>
+|
+* <small>May present as [[Extramedullary myeloid tumor|extramedullary]] disease ([[Extramedullary Myeloid Cell Tumor|Myeloid sarcoma]])</small>
+|-
+| colspan="2" |[[Myelodysplastic syndrome|Myelodysplastic syndromes]]<br>([[MDS]])<ref name="pmid24300826">{{cite journal |vauthors=Germing U, Kobbe G, Haas R, Gattermann N |title=Myelodysplastic syndromes: diagnosis, prognosis, and treatment |journal=Dtsch Arztebl Int |volume=110 |issue=46 |pages=783–90 |date=November 2013 |pmid=24300826 |pmc=3855821 |doi=10.3238/arztebl.2013.0783 |url=}}</ref><ref name="pmid26769228">{{cite journal |vauthors=Gangat N, Patnaik MM, Tefferi A |title=Myelodysplastic syndromes: Contemporary review and how we treat |journal=Am. J. Hematol. |volume=91 |issue=1 |pages=76–89 |date=January 2016 |pmid=26769228 |doi=10.1002/ajh.24253 |url=}}</ref>
+|
+* <small>Constitutional</small>
+* <small>[[Anemia]] related</small>
+* <small>[[Bleeding]]</small>
+* <small>[[Infection]]</small>
+|
+* <small>[[Pallor]]</small>
+* <small>[[Petechiae]]</small>
+* <small>[[Organomegaly]]</small>
+|[[Leukopenia|<small>↓</small>]]
+|<small>Variable</small>
+| -
+|<small>↓</small>
+|<small>↓</small>
+|<small>↓</small>
+|
+* <small>Macro-ovalocytes</small>
+* <small>Basophilic stippling</small>
+* [[Howell-Jolly body|<small>Howell-Jolly body</small>]]
+|[[Anemia|<small>↓</small>]]
+|<small>[[Thrombocytopenia|↓]]</small>
+|
+* <small>Hypercellular</small><small>/ normocellular [[bone marrow]] with [[Dysplastic change|dysplastic]] changes</small>
+|
+* <small>[[Cytogenetic analysis]]</small>
+* <small>[[Flow cytometry]]</small>
+|
+* <small>[[Leukemia]] transformation</small>
+* <small>Acquired pseudo-Pelger-Huët anomaly</small>
+|-
+| colspan="2" |[[Acute myeloid leukemia]] ([[AML]])<br>and related [[neoplasms]]<ref name="pmid3864727">{{cite journal |vauthors=Islam A, Catovsky D, Goldman JM, Galton DA |title=Bone marrow biopsy changes in acute myeloid leukaemia. I: Observations before chemotherapy |journal=Histopathology |volume=9 |issue=9 |pages=939–57 |date=September 1985 |pmid=3864727 |doi= |url=}}</ref><ref name="pmid17587881">{{cite journal |vauthors=Orazi A |title=Histopathology in the diagnosis and classification of acute myeloid leukemia, myelodysplastic syndromes, and myelodysplastic/myeloproliferative diseases |journal=Pathobiology |volume=74 |issue=2 |pages=97–114 |date=2007 |pmid=17587881 |doi=10.1159/000101709 |url=}}</ref>
+|
+* <small>Constitutional</small>
+* <small>[[Anemia]] related</small>
+* [[Bleeding|<small>Bleeding</small>]]
+* [[Bone pain|<small>Bone pain</small>]]
+* [[Joint pain|<small>Joint pain</small>]]
+* [[Infections|<small>Infections</small>]]
+|
+* <small>[[Infection]] related</small>
+* [[Pallor|<small>Pallor</small>]]
+* [[Chloroma|<small>Leukemia cutis</small>]]
+* <small>[[Bruising]] & [[Petechia|petechiae]]</small>
+* <small>[[Lymphadenopathy]]</small>
+* [[Hepatomegaly|<small>Hepatosplenomegaly</small>]]
+|<small>NL or ↑</small>
+|<small>↑</small>
+|<small>N/A</small>
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|
+* <small>↑ [[Potassium]]</small>
+* <small>↑ [[Uric acid]]</small>
+* <small>↑ [[Phosphorus]]</small>
+* <small>↓ [[Calcium]]</small>
+* <small>↑ [[LDH]]</small>
+|[[Anemia|<small>↓</small>]]
+|<small>[[Thrombocytopenia|↓]]</small>
+|
+* <small>Increased  immature[[myeloid]] [[cells]]</small>
+<small>with [[Dysplastic change|dysplasia]]</small>
+|
+* <small>[[Cytogenetic analysis]]</small>
+* <small>[[Flow cytometry]]</small>
+* <small>[[FISH]]</small>
+|
+* <small>Common in [[Down syndrome]]</small>
+|-
+| colspan="2" |[[Blastic plasmacytoid dendritic cell neoplasm|Blastic plasmacytoid<br>dendritic cell neoplasm]]<ref>{{Cite journal
+ | author = [[F. Julia]], [[T. Petrella]], [[M. Beylot-Barry]], [[M. Bagot]], [[D. Lipsker]], [[L. Machet]], [[P. Joly]], [[O. Dereure]], [[M. Wetterwald]], [[M. d'Incan]], [[F. Grange]], [[J. Cornillon]], [[G. Tertian]], [[E. Maubec]], [[P. Saiag]], [[S. Barete]], [[I. Templier]], [[F. Aubin]] & [[S. Dalle]]
+ | title = Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients
+ | journal = [[The British journal of dermatology]]
+ | volume = 169
+ | issue = 3
+ | pages = 579–586
+ | year = 2013
+ | month = September
+ | doi = 10.1111/bjd.12412
+ | pmid = 23646868
+}}</ref><ref>{{Cite journal
+ | author = [[Livio Pagano]], [[Caterina Giovanna Valentini]], [[Alessandro Pulsoni]], [[Simona Fisogni]], [[Paola Carluccio]], [[Francesco Mannelli]], [[Monia Lunghi]], [[Gianmatteo Pica]], [[Francesco Onida]], [[Chiara Cattaneo]], [[Pier Paolo Piccaluga]], [[Eros Di Bona]], [[Elisabetta Todisco]], [[Pellegrino Musto]], [[Antonio Spadea]], [[Alfonso D'Arco]], [[Stefano Pileri]], [[Giuseppe Leone]], [[Sergio Amadori]] & [[Fabio Facchetti]]
+ | title = Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study
+ | journal = [[Haematologica]]
+ | volume = 98
+ | issue = 2
+ | pages = 239–246
+ | year = 2013
+ | month = February
+ | doi = 10.3324/haematol.2012.072645
+ | pmid = 23065521
+}}</ref><ref>{{Cite journal
+ | author = [[Joseph D. Khoury]]
+ | title = Blastic Plasmacytoid Dendritic Cell Neoplasm
+ | journal = [[Current hematologic malignancy reports]]
+ | volume = 13
+ | issue = 6
+ | pages = 477–483
+ | year = 2018
+ | month = December
+ | doi = 10.1007/s11899-018-0489-z
+ | pmid = 30350260
+}}</ref><ref>{{Cite journal
+ | author = [[Shinichiro Sukegawa]], [[Mamiko Sakata-Yanagimoto]], [[Ryota Matsuoka]], [[Haruka Momose]], [[Yusuke Kiyoki]], [[Masayuki Noguchi]], [[Naoya Nakamura]], [[Rei Watanabe]], [[Manabu Fujimoto]], [[Yasuhisa Yokoyama]], [[Hidekazu Nishikii]], [[Takayasu Kato]], [[Manabu Kusakabe]], [[Naoki Kurita]], [[Naoshi Obara]], [[Yuichi Hasegawa]] & [[Shigeru Chiba]]
+ | title = &#91;Blastic plasmacytoid dendritic cell neoplasm accompanied by chronic myelomonocytic leukemia successfully treated with azacitidine&#93;
+ | journal = <nowiki>[[[Rinsho ketsueki] The Japanese journal of clinical hematology]]</nowiki>
+ | volume = 59
+ | issue = 12
+ | pages = 2567–2573
+ | year = 2018
+ | month =
+ | doi = 10.11406/rinketsu.59.2567
+ | pmid = 30626790
+}}</ref>
+|
+* <small>[[Cutaneous]] symptoms (brown/purple nodular lesions) on [[face]], [[scalp]], [[lower limb]] & [[trunk]]</small>
+|
+* <small>Brown/violaceous [[bruise]] like lesions</small>
+* <small>[[Lymphadenopathy]]</small>
+* <small>[[Splenomegaly]]</small>
+| <small>NL</small>
+| <small>↑</small>
+|
+|<small>NL</small>
+| <small>NL</small>
+| <small>NL</small>
+|
+* <small>[[Neutropenia]]</small>
+| [[Anemia|<small>↓</small>]]
+| <small>[[Thrombocytopenia|↓]]</small>
+|
+* <small>[[Malignant|Malignant cells]]</small>
+|
+* <small>[[Immunohistochemistry]] or [[flow cytometry]] for [[CD4]] & [[CD56]]</small>
+|
+* <small>TdT expression positive</small>
+* <small>May develop [[chronic myelomonocytic leukemia]] (CMML)</small>
+|-
+| rowspan="4" |[[Myelodysplastic]]<br>/[[Myeloproliferative neoplasm|myeloproliferative<br>neoplasms]] (MDS/MPN)
+|<small>[[Chronic myelomonocytic leukemia]] (CMML)</small><ref name="pmid27185207">{{cite journal |vauthors=Patnaik MM, Tefferi A |title=Chronic myelomonocytic leukemia: 2016 update on diagnosis, risk stratification, and management |journal=Am. J. Hematol. |volume=91 |issue=6 |pages=631–42 |date=June 2016 |pmid=27185207 |doi=10.1002/ajh.24396 |url=}}</ref>
+: <ref name="pmid22615103">{{cite journal |vauthors=Parikh SA, Tefferi A |title=Chronic myelomonocytic leukemia: 2012 update on diagnosis, risk stratification, and management |journal=Am. J. Hematol. |volume=87 |issue=6 |pages=610–9 |date=June 2012 |pmid=22615103 |doi=10.1002/ajh.23203 |url=}}</ref><ref name="pmid25869097">{{cite journal |vauthors=Benton CB, Nazha A, Pemmaraju N, Garcia-Manero G |title=Chronic myelomonocytic leukemia: Forefront of the field in 2015 |journal=Crit. Rev. Oncol. Hematol. |volume=95 |issue=2 |pages=222–42 |date=August 2015 |pmid=25869097 |pmc=4859155 |doi=10.1016/j.critrevonc.2015.03.002 |url=}}</ref>
+|
+* <small>Constitutional</small>
+* <small>[[Anemia]] related</small>
+* <small>[[Bleeding]]</small>
+* <small>[[Infections]]</small>
+* <small>[[Bone]] [[pain]]</small>
+* <small>[[Leukemia Cutis]]</small>
+|
+* <small>[[Organomegaly]]</small>
+* <small>[[Bruising]]</small>
+|↑
+| <small>< 20%</small>
+|
+|<small>NL</small>
+|<small>[[Eosinophilia|↑]]</small>
+|↑↑
+|
+* <small>↑ [[LDH]]</small>
+|<small>[[Anemia|↓]]</small>
+|<small>[[Thrombocytopenia|↓]]</small>
+|
+* <small>[[Myelodysplastic]] and [[myeloproliferative]] feature</small>
+|
+* <small>[[Cytogenetic analysis]]</small>
+* <small>[[Flow cytometry]]</small>
+|
+* <small>Overlapping of both, [[MDS]] and [[MPN]]</small>
+* <small>Absolute [[monocytosis]] > 1 × 10<sup>9</sup>/L (defining feature)</small>
+* <small>MD-CMML:</small><small>[[WBC]] ≤ 13 × 10<sup>9</sup>/L (FAB)</small>
+* <small> MP-CMML:</small><small>[[WBC]] > 13 × 10<sup>9</sup>/L</small> <small>(FAB)</small>
+|-
+|<small>[[Atypical chronic myeloid leukemia]] (aCML), [[BCR/ABL|BCR-ABL]]1-</small><ref name="pmid26637732">{{cite journal |vauthors=Dao KH, Tyner JW |title=What's different about atypical CML and chronic neutrophilic leukemia? |journal=Hematology Am Soc Hematol Educ Program |volume=2015 |issue= |pages=264–71 |date=2015 |pmid=26637732 |pmc=5266507 |doi=10.1182/asheducation-2015.1.264 |url=}}</ref><ref name="pmid22289493">{{cite journal |vauthors=Muramatsu H, Makishima H, Maciejewski JP |title=Chronic myelomonocytic leukemia and atypical chronic myeloid leukemia: novel pathogenetic lesions |journal=Semin. Oncol. |volume=39 |issue=1 |pages=67–73 |date=February 2012 |pmid=22289493 |pmc=3523950 |doi=10.1053/j.seminoncol.2011.11.004 |url=}}</ref>
+|<small>
+*Asymptomatic</small>
+* Constitutional
+* [[Hyperviscosity|Hyperviscosity]]  and/or [[anemia]] related
+* [[Bleeding|Bleeding]]
+* [[Infection|Infection]]
+|<small>
+* [[Splenomegaly]]</small>  (46–76%)
+* [[Purpura]]
+* [[Anemia]] related
+* [[Priapism]]
+|↑
+|<small><20%</small>
+| +
+|<small><2% of WBCs</small>
+|<small>N/A</small>
+|<small>N/A</small>
+|
+* <small>N/A</small>
+|<small>[[Anemia|↓]]</small>
+|<small>[[Thrombocytopenia|↓]]</small>
+|
+* <small>Granulocytic [[hyperplasia]] with prominent [[dysplasia]]</small>
+|
+* <small>[[Cytogenetic analysis]]</small>
+* <small>[[Flow cytometry]]</small>
+|
+* <small>Granulocytic [[dysplasia]] is prominent</small>
+* <small>Absence of ''[[BCR/ABL|BCR-ABL]]'' or ''PDGFRA'', [[PDGFRB|''PDGFRB'',]] or ''[[FGFR1]]'' rearrangements</small>
+* <small>[[WBC]] > 13 × 10<sup>9</sup>/L</small>
+|-
+|<small>[[Juvenile myelomonocytic leukemia (patient information)|Juvenile myelomonocytic leukemia (]]JMML)</small><ref name="pmid9226148">{{cite journal |vauthors=Aricò M, Biondi A, Pui CH |title=Juvenile myelomonocytic leukemia |journal=Blood |volume=90 |issue=2 |pages=479–88 |date=July 1997 |pmid=9226148 |doi= |url=}}</ref><ref name="pmid80255132">{{cite journal |vauthors=Hasle H |title=Myelodysplastic syndromes in childhood--classification, epidemiology, and treatment |journal=Leuk. Lymphoma |volume=13 |issue=1-2 |pages=11–26 |date=March 1994 |pmid=8025513 |doi=10.3109/10428199409051647 |url=}}</ref>
+|
+* <small>[[Infections]]</small>
+* <small>[[Anemia]] related</small>
+|
+* <small>[[Hepatosplenomegaly]]</small>
+* <small>[[Lymphadenopathy]]</small>
+* <small>[[Rash]]</small>
+|<small>[[Leukocytosis|↑]]</small>
+|<small>↑</small>
+|<small>N/A</small>
+|<small>N/A</small>
+|<small>N/A</small>
+|<small>[[Monocytosis|↑]]</small>
+|
+* <small>↓ [[Serum]] [[Iron]]</small>
+* <small>↑ [[Vitamin B12|B12]] levels</small>
+|<small>[[Anemia|↓]]</small>
+|<small>[[Thrombocytopenia|↓]]</small>
+|
+* <small>Hypercelluar with  ↑ [[myeloid cells]] in stages of maturation</small>
+|
+* <small>[[Cytogenetic analysis]]</small>
+* <small>[[Flow cytometry]]</small>
+|
+* <small>Polyclonal [[hypergammaglobulinemia]]</small>
+|-
+|<small>[[MDS]]/[[MPN]] with ring sideroblasts and [[thrombocytosis]] (MDS/MPN-RS-T)</small><ref name="pmid28188970">{{cite journal |vauthors=Patnaik MM, Tefferi A |title=Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis (RARS-T): 2017 update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=92 |issue=3 |pages=297–310 |date=March 2017 |pmid=28188970 |doi=10.1002/ajh.24637 |url=}}</ref><ref name="pmid30186759">{{cite journal |vauthors=Alshaban A, Padilla O, Philipovskiy A, Corral J, McAlice M, Gaur S |title=Lenalidomide induced durable remission in a patient with MDS/MPN-with ring sideroblasts and thrombocytosis with associated 5q- syndrome |journal=Leuk Res Rep |volume=10 |issue= |pages=37–40 |date=2018 |pmid=30186759 |doi=10.1016/j.lrr.2018.08.001 |url=}}</ref><ref name="pmid30524760">{{cite journal |vauthors=Bouchla A, Papageorgiou SG, Tsakiraki Z, Glezou E, Pavlidis G, Stavroulaki G, Bazani E, Foukas P, Pappa V |title=Plasmablastic Lymphoma in an Immunocompetent Patient with MDS/MPN with Ring Sideroblasts and Thrombocytosis-A Case Report |journal=Case Rep Hematol |volume=2018 |issue= |pages=2525070 |date=2018 |pmid=30524760 |pmc=6247723 |doi=10.1155/2018/2525070 |url=}}</ref>
+|
+* <small>Constitutional</small>
+* <small>[[Anemia]] related</small>
+* <small>[[Thrombosis]]</small>
+|
+* <small>Variable</small>
+|<small>NL or ↑</small>
+|<small>NL</small>
+| -
+|<small>NL</small>
+|<small>N/A</small>
+|<small>N/A</small>
+|
+* ↑ <small>[[Serum]] [[Iron]]</small>
+|<small>[[Anemia|↓]]</small>
+|<small>[[Thrombocytosis|↑]]</small>
+|
+* <small>Hypercellularity with [[dyserythropoiesis]] and increased [[megakaryocytes]]</small>
+|
+* <small>[[Cytogenetic analysis]]</small>
+* <small>[[Flow cytometry]]</small>
+|
+* <small>Large atypical [[megakaryocytes]]</small>
+* <small>Ringed [[sideroblasts]]</small>
+* <small>[[SF3B1]] [[mutation]]</small>
+|-
+| rowspan="3" |T-lymphoblastic leukemia/<br>lymphoma
+*
+*
+|<small>T-lymphoblastic leukemia/<br>lymphoma</small><ref name="pmid26276771">{{cite journal |vauthors=You MJ, Medeiros LJ, Hsi ED |title=T-lymphoblastic leukemia/lymphoma |journal=Am. J. Clin. Pathol. |volume=144 |issue=3 |pages=411–22 |date=September 2015 |pmid=26276771 |doi=10.1309/AJCPMF03LVSBLHPJ |url=}}</ref><ref name="pmid19284608">{{cite journal |vauthors=Patel KJ, Latif SU, de Calaca WM |title=An unusual presentation of precursor T cell lymphoblastic leukemia/lymphoma with cholestatic jaundice: case report |journal=J Hematol Oncol |volume=2 |issue= |pages=12 |date=March 2009 |pmid=19284608 |pmc=2663564 |doi=10.1186/1756-8722-2-12 |url=}}</ref><ref name="pmid24822133">{{cite journal |vauthors=Elreda L, Sandhu M, Sun X, Bekele W, Cohen AJ, Shah M |title=T-cell lymphoblastic leukemia/lymphoma: relapse 16 years after first remission |journal=Case Rep Hematol |volume=2014 |issue= |pages=359158 |date=2014 |pmid=24822133 |pmc=4005062 |doi=10.1155/2014/359158 |url=}}</ref>
+|
+* <small>Constitutional</small>
+* <small>[[Anemia]] Related</small>
+* <small>[[Bleeding]]</small>
+* <small>[[Superior vena cava syndrome]]</small>
+|
+* <small>[[Lymphadenopathy]]</small>
+* <small>[[Mediastinal mass]]</small>
+*  <small>[[Pleural effusions]]</small>
+* <small>[[Trachea|Tracheal]] obstruction</small>
+* <small>[[Pericardial effusions]]</small>
+|<small>↑</small>
+|<small>>25% [[Blast|blasts]] ([[Leukemia]])</small>
+<small><25% [[Blast|blasts]] ([[Lymphoma]])</small>
+|±
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|
+* ↑ <small>[[LDH]]</small>
+* <small>Positive for TdT</small>
+|<small>↓</small>
+|<small>↓</small>
+|
+* <small>Hypercelluarity with increased [[T cells]] precursors</small>
+|
+* <small>[[Cytogenetic analysis]]</small><small>[[Flow cytometry]]</small>
+* <small>[[FISH]]</small>
+|
+* <small>May involve [[brain]], [[skin]], and [[testes]].</small>
+|-
+|<small>Provisional entity: Natural killer (NK) cell lymphoblastic leukemia/lymph</small><ref name="pmid28868017">{{cite journal |vauthors=Sedick Q, Alotaibi S, Alshieban S, Naheet KB, Elyamany G |title=Natural Killer Cell Lymphoblastic Leukaemia/Lymphoma: Case Report and Review of the Recent Literature |journal=Case Rep Oncol |volume=10 |issue=2 |pages=588–595 |date=2017 |pmid=28868017 |doi=10.1159/000477843 |url=}}</ref>
+|
+* <small>Constitutional</small>
+* <small>[[Anemia]] Related</small>
+* <small>[[Bleeding]]</small>
+* <small>[[Superior vena cava syndrome]]</small>
+|
+* <small>[[Lymphadenopathy]]</small>
+* <small>[[Mediastinal mass]]</small>
+*  <small>[[Pleural effusions]]</small>
+* <small>[[Trachea|Tracheal]] obstruction</small>
+* <small>[[Pericardial effusions]]</small>
+|<small>↑</small>
+|<small>↑</small>
+|±
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|
+* ↑ <small>[[LDH]]</small>
+|<small>↓</small>
+|<small>↓</small>
+|
+* <small>N/A</small>
+|
+* <small>[[Cytogenetic analysis]]</small>
+* <small>[[FISH]]</small>
+* <small>[[Flow cytometry]]</small>
+|
+* <small>Similar to [[T-cell]] lymphoblastic [[leukemia]] but may have more aggressive clinical course.</small>
+* <small>[[Diagnosis]] is usually based on presence of [[CD56]] expression, and  [[T-cell]]-associated markers such as [[CD2]] and [[CD7]].</small>
+*  <small>[[B cell|B-cell]] markers are absent.</small>
+|-
+|<small>Provisional entity: Early T-cell precursor lymphoblastic leukemia</small><ref name="pmid26747249">{{cite journal |vauthors=Jain N, Lamb AV, O'Brien S, Ravandi F, Konopleva M, Jabbour E, Zuo Z, Jorgensen J, Lin P, Pierce S, Thomas D, Rytting M, Borthakur G, Kadia T, Cortes J, Kantarjian HM, Khoury JD |title=Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) in adolescents and adults: a high-risk subtype |journal=Blood |volume=127 |issue=15 |pages=1863–9 |date=April 2016 |pmid=26747249 |pmc=4915808 |doi=10.1182/blood-2015-08-661702 |url=}}</ref><ref name="pmid23695450">{{cite journal |vauthors=Haydu JE, Ferrando AA |title=Early T-cell precursor acute lymphoblastic leukaemia |journal=Curr. Opin. Hematol. |volume=20 |issue=4 |pages=369–73 |date=July 2013 |pmid=23695450 |pmc=3886681 |doi=10.1097/MOH.0b013e3283623c61 |url=}}</ref>
+|
+* <small>Constitutional</small>
+* <small>[[Anemia]] Related</small>
+* <small>[[Bleeding]]</small>
+* <small>[[Superior vena cava syndrome]]</small>
+|
+* <small>[[Lymphadenopathy]]</small>
+* <small>[[Mediastinal mass]]</small>
+*  <small>[[Pleural effusions]]</small>
+* <small>[[Trachea|Tracheal]] obstruction</small>
+* <small>[[Pericardial effusions]]</small>
+|<small>↑</small>
+|<small>↑</small>
+|±
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|<small>↑ or ↓</small>
+|
+* ↑ <small>[[LDH]]</small>
+|<small>↓</small>
+|<small>↓</small>
+|
+* <small>Hypercelluarity with increased [[T cells]] precursors</small>
+|
+* <small>[[Cytogenetic analysis]]</small>
+* <small>[[FISH]]</small>
+* <small>[[Flow cytometry]]</small>
+|
+* <small>Similar to [[T cell|T-cell]] lymphoblastic [[leukemia]] but is more aggressive clinically and cell are characterized by [[Flow cytometry|cytometry]] as [[CD1a]]<sup>−</sup>, [[CD8]]<sup>−</sup>, [[CD5]]<sup>−</sup> (dim), and positivity for 1 or more stem cell or [[myeloid]] [[antigens]].</small>
+* <small>[[Gene expression]] indicates more immature [[cells]] as compared to other subtypes of [[T cell|T-cell]] [[neoplasms]].</small>
+|}
 ==References==
 {{reflist|2}}