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! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings | ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings | ||
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! align="center" style="background:#DCDCDC;" |[[SLE]] | ! align="center" style="background:#DCDCDC;" |[[SLE]]<ref name="NewmanOwlia2013">{{cite journal|last1=Newman|first1=Kam|last2=Owlia|first2=Mohammad Bagher|last3=El-Hemaidi|first3=Ihab|last4=Akhtari|first4=Mojtaba|title=Management of immune cytopenias in patients with systemic lupus erythematosus — Old and new|journal=Autoimmunity Reviews|volume=12|issue=7|year=2013|pages=784–791|issn=15689972|doi=10.1016/j.autrev.2013.02.001}}</ref> | ||
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! align="center" style="background:#DCDCDC;" |[[Felty's syndrome|Felty syndrome]] | ! align="center" style="background:#DCDCDC;" |[[Felty's syndrome|Felty syndrome]]<ref name="LiatsosTsironi2018">{{cite journal|last1=Liatsos|first1=George D.|last2=Tsironi|first2=Ioanna|last3=Vassilopoulos|first3=Dimitrios|last4=Dourakis|first4=Spyridon|title=Severe pancytopenia and splenomegaly associated with felty's syndrome, both fully responsive solely to corticosteroids|journal=Clinical Case Reports|volume=6|issue=3|year=2018|pages=509–512|issn=20500904|doi=10.1002/ccr3.1396}}</ref> | ||
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! align="center" style="background:#DCDCDC;" |[[Wiskott-Aldrich syndrome|Wiskott Aldrich syndrome]] | ! align="center" style="background:#DCDCDC;" |[[Wiskott-Aldrich syndrome|Wiskott Aldrich syndrome]]<ref name="pmid1960605">{{cite journal |vauthors=Brochstein JA, Gillio AP, Ruggiero M, Kernan NA, Emanuel D, Laver J, Small T, O'Reilly RJ |title=Marrow transplantation from human leukocyte antigen-identical or haploidentical donors for correction of Wiskott-Aldrich syndrome |journal=J. Pediatr. |volume=119 |issue=6 |pages=907–12 |date=December 1991 |pmid=1960605 |doi= |url=}}</ref> | ||
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! align="center" style="background:#DCDCDC;" |[[GATA2]] deficiency | ! align="center" style="background:#DCDCDC;" |[[GATA2]] deficiency<ref name="SanyiJaye2018">{{cite journal|last1=Sanyi|first1=Allen|last2=Jaye|first2=David L.|last3=Rosand|first3=Cecilia B.|last4=Box|first4=Amanda|last5=Shanmuganathan|first5=Chandrakasan|last6=Waller|first6=Edmund K.|title=Diagnosis of GATA2 haplo-insufficiency in a young woman prompted by pancytopenia with deficiencies of B-cell and dendritic cell development|journal=Biomarker Research|volume=6|issue=1|year=2018|issn=2050-7771|doi=10.1186/s40364-018-0127-x}}</ref> | ||
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! align="center" style="background:#DCDCDC;" |[[Hemophagocytic lymphohistiocytosis]] | ! align="center" style="background:#DCDCDC;" |[[Hemophagocytic lymphohistiocytosis]]<ref name="Larroche2012">{{cite journal|last1=Larroche|first1=Claire|title=Hemophagocytic lymphohistiocytosis in adults: Diagnosis and treatment|journal=Joint Bone Spine|volume=79|issue=4|year=2012|pages=356–361|issn=1297319X|doi=10.1016/j.jbspin.2011.10.015}}</ref> | ||
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! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |GI disorders | ! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |GI disorders | ||
! align="center" style="background:#DCDCDC;" |[[Portal hypertension]]/[[cirrhosis]] | ! align="center" style="background:#DCDCDC;" |[[Portal hypertension]]/[[cirrhosis]]<ref name="SarinKhanna2014">{{cite journal|last1=Sarin|first1=Shiv K.|last2=Khanna|first2=Rajeev|title=Non-cirrhotic Portal Hypertension|journal=Clinics in Liver Disease|volume=18|issue=2|year=2014|pages=451–476|issn=10893261|doi=10.1016/j.cld.2014.01.009}}</ref><ref name="AksoyKoklu2015">{{cite journal|last1=Aksoy|first1=Evrim K.|last2=Koklu|first2=Seyfettin|title=Albendazole-Induced Cirrhosis Decompensation and Pancytopenia|journal=Annals of Pharmacotherapy|volume=49|issue=8|year=2015|pages=945–946|issn=1060-0280|doi=10.1177/1060028015590178}}</ref> | ||
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* Splenomegally | * Splenomegally | ||
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! align="center" style="background:#DCDCDC;" |[[Glycogen storage disease|Storage diseases]] (eg, [[Gaucher's disease|Gaucher]]) | ! align="center" style="background:#DCDCDC;" |[[Glycogen storage disease|Storage diseases]] (eg, [[Gaucher's disease|Gaucher]])<ref name="Düzenli KarÖzdemir2018">{{cite journal|last1=Düzenli Kar|first1=Yeter|last2=Özdemir|first2=Zeynep C.|last3=Kiral|first3=Eylem|last4=Kiliç Yildirim|first4=Gonca|last5=Dinleyici|first5=Ener Ç.|last6=Bör|first6=Özcan|title=Hemophagocytic Lymphohystiocytosis Associated With Type Ia Glycogen Storage Disease|journal=Journal of Pediatric Hematology/Oncology|year=2018|pages=1|issn=1077-4114|doi=10.1097/MPH.0000000000001208}}</ref> | ||
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* Splenomegaly | * Splenomegaly | ||
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! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings | ! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings | ||
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! align="center" style="background:#DCDCDC;" |[[Sepsis]] | ! align="center" style="background:#DCDCDC;" |[[Sepsis]]<ref name="TendasNiscola2010">{{cite journal|last1=Tendas|first1=Andrea|last2=Niscola|first2=Pasquale|last3=Dentamaro|first3=Teresa|last4=Cupelli|first4=Luca|last5=Di Matteo|first5=Gigliola|last6=Finocchi|first6=Andrea|last7=Siniscalchi|first7=Agostina|last8=Fratoni|first8=Stefano|last9=Scimò|first9=Teresa|last10=Scaramucci|first10=Laura|last11=Giovannini|first11=Marco|last12=Ales|first12=Micaela|last13=Pio Perrotti|first13=Alessio|last14=de Fabritiis|first14=Paolo|title=Pancytopenia and severe sepsis in an adult case of congenital X-linked agammaglobulinemia (XLA)|journal=Annals of Hematology|volume=89|issue=9|year=2010|pages=949–951|issn=0939-5555|doi=10.1007/s00277-009-0891-7}}</ref> | ||
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* Bone marrow infiltration | * Bone marrow infiltration | ||
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! align="center" style="background:#DCDCDC;" |[[Infection|Viral infection]] such as [[Human Immunodeficiency Virus (HIV)|HIV]], [[hepatitis]], [[Epstein Barr virus|Epstein-Barr virus]] | ! align="center" style="background:#DCDCDC;" |[[Infection|Viral infection]] such as [[Human Immunodeficiency Virus (HIV)|HIV]], [[hepatitis]], [[Epstein Barr virus|Epstein-Barr virus]]<ref name="Santiago-RodríguezMayor2015">{{cite journal|last1=Santiago-Rodríguez|first1=Eduardo|last2=Mayor|first2=Angel|last3=Fernández-Santos|first3=Diana|last4=Hunter-Mellado|first4=Robert|title=Profile of HIV-Infected Hispanics with Pancytopenia|journal=International Journal of Environmental Research and Public Health|volume=13|issue=1|year=2015|pages=38|issn=1660-4601|doi=10.3390/ijerph13010038}}</ref> | ||
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* Marrow suppression | * Marrow suppression |
Revision as of 20:38, 10 September 2018
Differentiating Pancytopenia from Other Diseases
- There are no other mimickers of pancytopenia, as the diagnosis can be unequivocally established by laboratory testing (complete blood count).
Category | Condition | Etiology | Mechanism | Congenital | Acquried | Clinical manifestations | Para−clinical findings | Gold standard | Associated findings | |||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Demography | History | Symptoms | Signs | |||||||||||||||||||||||
Lab Findings | ||||||||||||||||||||||||||
Appearance | Fever | Bleeding | BP | Splenomegaly | Jaundice | Other | CBC | PBS | Bone marrow exam | PT | PTT | UA | ||||||||||||||
Bone marrow infiltration | Bone marrow failure | Destruction/
sequestration/ redistribution |
Plt | HB | WBC | |||||||||||||||||||||
Hematologic disorders | Myelodysplastic syndrome[1] |
|
+ | + | − | ± | + | Elderly | Exposure to | + | + | Nl | + | + | ↓ | ↓ | ↓ |
|
|
Nl | Nl | Nl | Bone marrow examination + clinical manifestation |
| ||
Malignancies such as:[2][3] |
|
+ | + | + | ± | ± | Any, more in adults |
|
+ | + | Nl | + | − | ↓ | ↓ | ↓ |
|
↑ | ↑ | Bone marrow examination |
| |||||
Myelofibrosis[4] |
|
+ | + | − | + | + | Average 60 years old |
|
|
+ | + | Nl | + | - | ↓ | ↓ | ↓ |
|
↑ | ↑ | Bone marrow examination |
| ||||
Fanconi anemia[5] | ||||||||||||||||||||||||||
Condition | Etiology | Bone marrow infiltration | Bone marrow failure | Destruction/
sequestration/ redistribution |
Congenital | Acquried | Demography | History | Appearance | Fever | Bleeding | BP | Splenomegaly | Jaundice | Other signs | Plt | HB | WBC | PBS | Bone marrow exam | PT | PTT | UA | Gold standard | Associated findings | |
Aplastic anemia[6] |
|
− | + | − | ± | ± | Biphasic (the young and the elderly) |
|
− | + | Nl | − | − | ↓ | ↓ | ↓ |
|
|
↑ | ↑ | Nl | Bone marrow examination +
laboratory findings |
||||
Paroxysmal nocturnal hemoglobinuria[7] |
|
+ | + | − | − | + | Any age
(usually younger adults) |
|
|
− | − | Nl | − | − |
|
↓/Nl | ↓ | ↓/Nl |
|
Nl | Nl | Flow cytometry | ||||
Disseminated intravascular coagulation[8] | Consumption | |||||||||||||||||||||||||
Dyskeratosis congenital/telomere biology disorders[9] | ||||||||||||||||||||||||||
Shwachman-Diamond syndrome[10] | + | |||||||||||||||||||||||||
Immunology/
Rheumatology |
Condition | Etiology | Bone marrow infiltration | Bone marrow failure | Destruction/
sequestration/ redistribution |
Congenital | Acquried | Demography | History | Appearance | Fever | Bleeding | BP | Splenomegaly | Jaundice | Other signs | Plt | HB | WBC | PBS | Bone marrow exam | PT | PTT | UA | Gold standard | Associated findings |
SLE[11] | ||||||||||||||||||||||||||
Felty syndrome[12] | ||||||||||||||||||||||||||
Wiskott Aldrich syndrome[13] | + | |||||||||||||||||||||||||
GATA2 deficiency[14] | + | |||||||||||||||||||||||||
Hemophagocytic lymphohistiocytosis[15] | + | |||||||||||||||||||||||||
GI disorders | Portal hypertension/cirrhosis[16][17] |
|
||||||||||||||||||||||||
Storage diseases (eg, Gaucher)[18] |
|
+ | + | |||||||||||||||||||||||
Infections | Condition | Etiology | Bone marrow infiltration | Bone marrow failure | Destruction/
sequestration/ redistribution |
Congenital | Acquried | Demography | History | Appearance | Fever | Bleeding | BP | Splenomegaly | Jaundice | Other signs | Plt | HB | WBC | PBS | Bone marrow exam | PT | PTT | UA | Gold standard | Associated findings |
Sepsis[19] |
|
|||||||||||||||||||||||||
Viral infection such as HIV, hepatitis, Epstein-Barr virus[20] |
|
+ | + | |||||||||||||||||||||||
Nutritional | Megaloblastic anemia |
|
+ | |||||||||||||||||||||||
Excessive alcohol |
|
|||||||||||||||||||||||||
Other nutritional deficiency such as copper deficiency, zinc toxicity |
|
|||||||||||||||||||||||||
Malnutrition |
|
|||||||||||||||||||||||||
Medications | Medications such as:
|
|
+ | |||||||||||||||||||||||
Category | Condition | Etiology | Bone marrow infiltration | Bone marrow failure | Destruction/
sequestration/ redistribution |
Congenital | Acquried | Demography | History | Appearance | Fever | Bleeding | BP | Splenomegaly | Jaundice | Other signs | Plt | HB | WBC | PBS | Bone marrow exam | PT | PTT | UA | Gold standard | Associated findings |
- ↑ Natelson, Ethan A.; Pyatt, David (2013). "Acquired Myelodysplasia or Myelodysplastic Syndrome: Clearing the Fog". Advances in Hematology. 2013: 1–11. doi:10.1155/2013/309637. ISSN 1687-9104.
- ↑ Oshima, Yasuo; Yuji, Koichiro; Tanimoto, Tetsuya; Hinomura, Yasushi; Tojo, Arinobu (2013). "Association between Acute Myelogenous Leukemia and Thrombopoietin Receptor Agonists in Patients with Immune Thrombocytopenia". Internal Medicine. 52 (19): 2193–2201. doi:10.2169/internalmedicine.52.0324. ISSN 0918-2918.
- ↑ Oshima Y, Yuji K, Tanimoto T, Hinomura Y, Tojo A (2013). "Association between acute myelogenous leukemia and thrombopoietin receptor agonists in patients with immune thrombocytopenia". Intern. Med. 52 (19): 2193–201. PMID 24088751.
- ↑ Bae, E.; Park, C.-J.; Cho, Y.-U.; Seo, E.-J.; Chi, H.-S.; Jang, S.; Lee, K.-H.; Lee, J.-H.; Lee, J.-H.; Suh, J.-J.; Im, H.-J. (2013). "Differential diagnosis of myelofibrosis based on WHO 2008 criteria: Acute panmyelosis with myelofibrosis, acute megakaryoblastic leukemia with myelofibrosis, primary myelofibrosis and myelodysplastic syndrome with myelofibrosis". International Journal of Laboratory Hematology. 35 (6): 629–636. doi:10.1111/ijlh.12101. ISSN 1751-5521.
- ↑ Zhang, Qing-Shuo; Benedetti, Eric; Deater, Matthew; Schubert, Kathryn; Major, Angela; Pelz, Carl; Impey, Soren; Marquez-Loza, Laura; Rathbun, R. Keaney; Kato, Shigeaki; Bagby, Grover C.; Grompe, Markus (2015). "Oxymetholone Therapy of Fanconi Anemia Suppresses Osteopontin Transcription and Induces Hematopoietic Stem Cell Cycling". Stem Cell Reports. 4 (1): 90–102. doi:10.1016/j.stemcr.2014.10.014. ISSN 2213-6711.
- ↑ Dolberg, Osnat Jarchowsky; Levy, Yair (2014). "Idiopathic aplastic anemia: Diagnosis and classification". Autoimmunity Reviews. 13 (4–5): 569–573. doi:10.1016/j.autrev.2014.01.014. ISSN 1568-9972.
- ↑ Boyer, Thomas; Grardel, Nathalie; Copin, Marie-Christine; Roumier, Christophe; Touzart, Aurore; Buchdahl, Anne-Laure; Corm, Sélim; de Latour, Régis Peffault; Preudhomme, Claude; Terriou, Louis (2015). "Paroxysmal nocturnal hemoglobinuria (PNH) and T cell large granular lymphocyte (LGL) leukemia—an unusual association: another cause of cytopenia in PNH". Annals of Hematology. 94 (10): 1759–1760. doi:10.1007/s00277-015-2439-3. ISSN 0939-5555.
- ↑ Hossain, Nazli; Paidas, Michael J. (2013). "Disseminated intravascular coagulation". Seminars in Perinatology. 37 (4): 257–266. doi:10.1053/j.semperi.2013.04.008. ISSN 0146-0005.
- ↑ Paiva, Raquel M.A.; Calado, Rodrigo T. (2014). "Telomere Dysfunction and Hematologic Disorders". 125: 133–157. doi:10.1016/B978-0-12-397898-1.00006-2. ISSN 1877-1173.
- ↑ Gokce, Muge; Tuncer, Murat; Cetin, Mualla; Gumruk, Fatma (2012). "Molecular Diagnosis of Shwachman-Diamond Syndrome Presenting with Pancytopenia at an Early Age: The First Report from Turkey". Indian Journal of Hematology and Blood Transfusion. 29 (3): 161–163. doi:10.1007/s12288-012-0163-x. ISSN 0971-4502.
- ↑ Newman, Kam; Owlia, Mohammad Bagher; El-Hemaidi, Ihab; Akhtari, Mojtaba (2013). "Management of immune cytopenias in patients with systemic lupus erythematosus — Old and new". Autoimmunity Reviews. 12 (7): 784–791. doi:10.1016/j.autrev.2013.02.001. ISSN 1568-9972.
- ↑ Liatsos, George D.; Tsironi, Ioanna; Vassilopoulos, Dimitrios; Dourakis, Spyridon (2018). "Severe pancytopenia and splenomegaly associated with felty's syndrome, both fully responsive solely to corticosteroids". Clinical Case Reports. 6 (3): 509–512. doi:10.1002/ccr3.1396. ISSN 2050-0904.
- ↑ Brochstein JA, Gillio AP, Ruggiero M, Kernan NA, Emanuel D, Laver J, Small T, O'Reilly RJ (December 1991). "Marrow transplantation from human leukocyte antigen-identical or haploidentical donors for correction of Wiskott-Aldrich syndrome". J. Pediatr. 119 (6): 907–12. PMID 1960605.
- ↑ Sanyi, Allen; Jaye, David L.; Rosand, Cecilia B.; Box, Amanda; Shanmuganathan, Chandrakasan; Waller, Edmund K. (2018). "Diagnosis of GATA2 haplo-insufficiency in a young woman prompted by pancytopenia with deficiencies of B-cell and dendritic cell development". Biomarker Research. 6 (1). doi:10.1186/s40364-018-0127-x. ISSN 2050-7771.
- ↑ Larroche, Claire (2012). "Hemophagocytic lymphohistiocytosis in adults: Diagnosis and treatment". Joint Bone Spine. 79 (4): 356–361. doi:10.1016/j.jbspin.2011.10.015. ISSN 1297-319X.
- ↑ Sarin, Shiv K.; Khanna, Rajeev (2014). "Non-cirrhotic Portal Hypertension". Clinics in Liver Disease. 18 (2): 451–476. doi:10.1016/j.cld.2014.01.009. ISSN 1089-3261.
- ↑ Aksoy, Evrim K.; Koklu, Seyfettin (2015). "Albendazole-Induced Cirrhosis Decompensation and Pancytopenia". Annals of Pharmacotherapy. 49 (8): 945–946. doi:10.1177/1060028015590178. ISSN 1060-0280.
- ↑ Düzenli Kar, Yeter; Özdemir, Zeynep C.; Kiral, Eylem; Kiliç Yildirim, Gonca; Dinleyici, Ener Ç.; Bör, Özcan (2018). "Hemophagocytic Lymphohystiocytosis Associated With Type Ia Glycogen Storage Disease". Journal of Pediatric Hematology/Oncology: 1. doi:10.1097/MPH.0000000000001208. ISSN 1077-4114.
- ↑ Tendas, Andrea; Niscola, Pasquale; Dentamaro, Teresa; Cupelli, Luca; Di Matteo, Gigliola; Finocchi, Andrea; Siniscalchi, Agostina; Fratoni, Stefano; Scimò, Teresa; Scaramucci, Laura; Giovannini, Marco; Ales, Micaela; Pio Perrotti, Alessio; de Fabritiis, Paolo (2010). "Pancytopenia and severe sepsis in an adult case of congenital X-linked agammaglobulinemia (XLA)". Annals of Hematology. 89 (9): 949–951. doi:10.1007/s00277-009-0891-7. ISSN 0939-5555.
- ↑ Santiago-Rodríguez, Eduardo; Mayor, Angel; Fernández-Santos, Diana; Hunter-Mellado, Robert (2015). "Profile of HIV-Infected Hispanics with Pancytopenia". International Journal of Environmental Research and Public Health. 13 (1): 38. doi:10.3390/ijerph13010038. ISSN 1660-4601.