Sandbox:Preeti: Difference between revisions

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{|
{|
! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Category
! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Category
!
! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
Line 13: Line 14:
! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated findings
! rowspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated findings
|-
|-
!
! colspan="9" align="center" style="background:#4479BA; color: #FFFFFF;" + |Lab findings
! colspan="9" align="center" style="background:#4479BA; color: #FFFFFF;" + |Lab findings
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Imaging
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" + |Imaging
|-
|-
!
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Demography
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Demography
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |History
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |History
Line 28: Line 31:
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Other
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Other
|-
|-
!
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Inherited
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Inherited
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Acquired
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Acquired
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! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Plt
|-
|-
! colspan="29" align="left" style="background:#4479BA; color: #FFFFFF;" + |HL
! colspan="30" align="left" style="background:#4479BA; color: #FFFFFF;" + |HL
|-
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" + |
! align="center" style="background:#4479BA; color: #FFFFFF;" + |
!
! align="center" style="background:#DCDCDC;" + |Hodgkin's Lymphoma
! align="center" style="background:#DCDCDC;" + |Hodgkin's Lymphoma
| align="center" style="background:#F5F5F5;" + |
| align="center" style="background:#F5F5F5;" + |
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| align="center" style="background:#F5F5F5;" + |
| align="center" style="background:#F5F5F5;" + |
|-
|-
! colspan="29" align="left" style="background:#4479BA; color: #FFFFFF;" + |NHL
! colspan="30" align="left" style="background:#4479BA; color: #FFFFFF;" + |NHL
|-
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" + |B Cell Lymphoma
! align="center" style="background:#4479BA; color: #FFFFFF;" + |B Cell Lymphoma
!
! align="center" style="background:#DCDCDC;" + |Precursor B-cell lymphoblastic leukemia/lymphoma
! align="center" style="background:#DCDCDC;" + |Precursor B-cell lymphoblastic leukemia/lymphoma
|
|
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|-
|-
!
!
|
|[[Follicular lymphoma]]
|[[Follicular lymphoma]]
|
|
Line 155: Line 162:
|-
|-
!
!
|
|Mantle cell lymphoma
|Mantle cell lymphoma
|
|
* Mature B-cell Lymphoma
* [[CD5 (protein)|CD5]] positive antigen in pregerminal center of B-cell
|
* [[Chromosomal translocation]] at '''t(11:14)'''
** Over-express [[cyclin D1]]
|
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* Stage IV disease
* B symptoms,
* Generalized lymphadenopathy
* Abdominal distention
* Fatigue
* Night sweats
* Weight Loss
* Extranodal involvement of gastrointtestinal (GI) tract, lungs, and central nervous system (CNS)
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* Generalized lymphadenopathy
* Hepato-splenomegaly
* Mental Retardation
* Less commonly
** Palpable masses in skin, breast, and salivary glands
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|CBC
* Anemia and cytopenias are secondary to bone marrow infiltration
* Lymphocytosis > 4000/µL
* Elevated LDH
*
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* CD5<sup>+</sup>
* B-cell antigen positive
** CD19
** CD20
** CD22
* Cyclin D1 is overexpressed.
|
|
* Germinal centers filled by small-to-medium atypical lymphocytes.
* Nodular appearance may be evident from expansion of the mantle zone in 30-50% of patients early in the disease.
|
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|-
|-
!
!
|
|Diffuse large B cell lymphoma
|Diffuse large B cell lymphoma
|
|
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|-
|-
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Category
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Category
!
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
Line 264: Line 301:
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated findings
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Associated findings
|-
|-
!
!
!
! align="center" style="background:#DCDCDC;" + |[[Burkitt lymphoma]]
! align="center" style="background:#DCDCDC;" + |[[Burkitt lymphoma]]
Line 326: Line 364:
|-
|-
!
!
|
|B cell chronic lymphocytic leukemia/small lymphocytic lymphoma
|B cell chronic lymphocytic leukemia/small lymphocytic lymphoma
|
|
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!
!
|Marginal zone lymphoma
|Marginal zone lymphoma
|Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type
|
|
* Mature B-cell Lymphoma
* Mature B-cell Lymphoma
Line 387: Line 427:
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|-
|-
!
|Lymphoplasmacytic lymphoma (Waldenstrom’s macroglobulinemia)
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* Mature B-cell Lymphoma
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|[[Splenic marginal zone lymphoma]]
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|
* Clonal rearrangements of the [[immunoglobulin]] genes (heavy and light chains) .
** Deletion 7q21-32
** Translocations of the CDK6 gene located at 7q21.
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* [[CD20]]
* [[CD79a]]
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* [[B-cells]] replace the normal [[white pulp]] of the [[spleen]].
* The neoplastic cells compromise
** Small [[lymphocytes]]
** Transformed blasts
* S[[Sinus|inus]] invasion
* Epithelial histocytes
* Plasmacytic differentiation of neoplastic cells.
* '''Splenic Hilar Lymph Nodes'''
** Involved hilar [[lymph nodes]] adjacent to the [[spleen]] show an effaced architecture without preservation of the [[marginal zone]] seen in the spleen
* '''Bone Marrow Biopsy'''
** Splenic marginal zone lymphoma in [[bone marrow]] displays a nodular pattern with morphology similar to what is observed in the splenic [[hilar]] [[lymph nodes]].
|
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|-
| rowspan="25" |B cell lymphoma
|[[Mantle cell lymphoma]]
|
* [[CD5 (protein)|CD5]] positive antigen in pregerminal center of B-cell
* [[Chromosomal translocation]] at '''t(11:14)'''
** Over-express [[cyclin D1]]
|
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|
|
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|-
|
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|
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* Stage IV disease
* B symptoms,
* Generalized lymphadenopathy
* Abdominal distention
* Fatigue
* Night sweats
* Weight Loss
* Extranodal involvement of gastrointtestinal (GI) tract, lungs, and central nervous system (CNS)
|
|
|
|
|
* Generalized lymphadenopathy
* Hepato-splenomegaly
* Mental Retardation
* Less commonly
** Palpable masses in skin, breast, and salivary glands
|
|
|
|
|CBC
* Anemia and cytopenias are secondary to bone marrow infiltration
* Lymphocytosis > 4000/µL
* Elevated LDH
*
|
|
|
|
|
|
* CD5<sup>+</sup>
* B-cell antigen positive
** CD19
** CD20
** CD22
* Cyclin D1 is overexpressed.
|
* Germinal centers filled by small-to-medium atypical lymphocytes.
* Nodular appearance may be evident from expansion of the mantle zone in 30-50% of patients early in the disease.
|
|
|
|
|-
|[[Nodal marginal zone B-cell lymphoma]]  
|[[Nodal marginal zone B-cell lymphoma]]  
|
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|-
|-
|[[Splenic marginal zone lymphoma]]
|
|
* Clonal rearrangements of the [[immunoglobulin]] genes (heavy and light chains) .
|
** Deletion 7q21-32
|Lymphoplasmacytic lymphoma (Waldenstrom’s macroglobulinemia)
** Translocations of the CDK6 gene located at 7q21.
|
* Mature B-cell Lymphoma
|
|
|
|
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* [[CD20]]
* [[CD79a]]
|
|
* [[B-cells]] replace the normal [[white pulp]] of the [[spleen]].
* The neoplastic cells compromise
** Small [[lymphocytes]]
** Transformed blasts
* S[[Sinus|inus]] invasion
* Epithelial histocytes
* Plasmacytic differentiation of neoplastic cells.
* '''Splenic Hilar Lymph Nodes'''
** Involved hilar [[lymph nodes]] adjacent to the [[spleen]] show an effaced architecture without preservation of the [[marginal zone]] seen in the spleen
* '''Bone Marrow Biopsy'''
** Splenic marginal zone lymphoma in [[bone marrow]] displays a nodular pattern with morphology similar to what is observed in the splenic [[hilar]] [[lymph nodes]].
|
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|-
|-
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|[[Hairy cell leukemia]]
|[[Hairy cell leukemia]]
|
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|-
|-
|[[Multiple myeloma|Plasma cell myeloma]]
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|Precursor T-cell lymphoblastic leukemia/lymphoma
|
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* '''Precursor T-cell Lymphomas'''
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|-
|[[Chronic lymphocytic leukemia]] / [[Chronic lymphocytic leukemia|small lymphocytic lymphoma]]
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|-
|[[Monoclonal B-cell lymphocytosis]]
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|Adult T cell leukemia/lymphoma
|-
|[[B-cell prolymphocytic leukemia]]
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|-
|-
|[[Waldenström's macroglobulinemia|Waldenström's macroglubulinemia]]
|T cell lymphoma
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|Anaplastic large cell lymphoma
|
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|-
|[[Monoclonal gammopathy of undetermined significance]] (MGUS)
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|-
|-
|[[Heavy chain disease]]
|
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|Cutaneous T-cell lymphoma
|[[Mycosis fungoides]] / [[Sézary syndrome]]
|
|
* The [[tumor]] [[Cell (biology)|cells]] originate from [[memory T cells]] or [[skin]] homing [[CD4+ T cells]] expressing [[cutaneous]] [[lymphocyte]] [[antigen]] (CLA) and [[chemokine]] [[receptors]] [[CCR4]]<nowiki/>and CCR7.
* It is understood that cutaneous t cell lymphoma (maycosis fungoides, Sezary sydrome ) is the result of malignant T cell that derived from a mature CD41 CD45RO1 memory T cells.
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* [[Epidermis (skin)|Epidermal]] [[atrophy]] or poikiloderma
* Generalized [[itching]]([[pruritus]])
* [[Pain]] in the affected area of the skin.
* [[Insomnia]]
* Red ([[erythematous]]) patches scattered over the [[skin]] of the [[trunk]] and the [[extremities]]
* [[Weight loss]]
* [[Lymphadenopathy]]
* [[Malaise]] and [[fatigue]]
* [[Anemia]]
* May progress to [[Sezary syndrome]] (Skin involvement plus hematogenous dissemination)
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* Cutaneous manifestaions
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|Solitary plasmacytoma of bone
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|Extraosseous plasmacytoma
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| rowspan="6" |Peripheral T-cell lymphoma
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|Subcutaneous panniculitis-like T-cell lymphoma
|-
|Monoclonal immunoglobulin deposition diseases
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|Extranodal marginal zone lymphoma of [[MALT lymphoma|mucosa-associated lymphoid tissue]] (MALT lymphoma)
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|Hepatosplenic gamma-delta T-cell lymphoma
|Large B-cell lymphoma with IRF4 rearrangement
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|[[Primary cutaneous follicle centre lymphoma|Primary cutaneous follicle center lymphoma]]
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|T-cell/histiocyte-rich large B-cell lymphoma
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|[[Lymphomatoid granulomatosis]]
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|[[Primary mediastinal large B-cell lymphoma|Primary mediastinal (thymic) large B-cell lymphoma]]
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|[[Intravascular large B-cell lymphoma]]
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|ALK1 large B-cell lymphoma
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|Plasmablastic lymphoma
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|[[Primary effusion lymphoma]]
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|High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements
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|-
| rowspan="6" |T cell lymphoma
|[[Mycosis fungoides]] / [[Sézary syndrome]]
|
* The [[tumor]] [[Cell (biology)|cells]] originate from [[memory T cells]] or [[skin]] homing [[CD4+ T cells]] expressing [[cutaneous]] [[lymphocyte]] [[antigen]] (CLA) and [[chemokine]] [[receptors]] [[CCR4]]<nowiki/>and CCR7.
* It is understood that cutaneous t cell lymphoma (maycosis fungoides, Sezary sydrome ) is the result of malignant T cell that derived from a mature CD41 CD45RO1 memory T cells.
|
|
|
|
|
* [[Epidermis (skin)|Epidermal]] [[atrophy]] or poikiloderma
* Generalized [[itching]]([[pruritus]])
* [[Pain]] in the affected area of the skin.
* [[Insomnia]]
* Red ([[erythematous]]) patches scattered over the [[skin]] of the [[trunk]] and the [[extremities]]
* [[Weight loss]]
* [[Lymphadenopathy]]
* [[Malaise]] and [[fatigue]]
* [[Anemia]]
* May progress to [[Sezary syndrome]] (Skin involvement plus hematogenous dissemination)
|
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* Cutaneous manifestaions
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Line 1,283: Line 821:
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|-
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|Enteropathy-type intestinal T-cell lymphoma
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Line 1,312: Line 851:
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|-
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|Extranodal T-cell lymphoma, nasal type
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Line 1,341: Line 881:
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|-
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|Angioimmunoblastic T-cell lymphoma
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Line 1,369: Line 910:
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|-
|-
|[[T-cell granular lymphocytic leukemia]]
|
* Disregulation of signaling pathways:
** FAS/FAS-L
** Phosphatidylinositol-3 kinase (PI3K),
** Mitogen-activated proteinkinase/extracellular signal-regulated kinase (MAPK/ERK)
|
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|
Symptoms of T-cell large granular lymphocyte leukemia may include the following:
* Generalised weakness and [[Fatigue (physical)|fatigue]]
* [[Anorexia]]
* Joint pain
* Night sweating
* [[Epistaxis]]
* [[Bone pain]]
* [[Dyspnea]]
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* Usually appear pale and malnourished.
* Cardiac flow murmur
* High-grade fever
* [[Hepatomegaly]]
* [[Splenomegaly]]
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* Neutropenia
* Anemia
* Hypergammaglobulinemia
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* [[CD3]]+
* [[TCR]]αβ+
* [[CD4]]-
* [[CD8]]+
|
:* Clonal rearrangements of the [[T-cell receptor]] (TCR) gene
:* Chronic (> 6 months) elevation in large granular lymphocytes (LGLs) in the peripheral blood
:* Large granular lymphocyte count greater than 2.0 × 109/L
:* Lymphocytosis (typically 2-20x109/L)
|
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|Peripheral T-cell lymphoma, unspecified
|
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|-
|[[Subcutaneous panniculitis-like T-cell lymphoma]]
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Revision as of 20:11, 28 December 2018


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

Category Disease Etiology Mechanism Clinical manifestations Paraclinical findings Gold standard Associated findings
Lab findings Imaging
Demography History Symptoms Signs CBC PBS LFT PT/PTT Immunochemistry Histopathology Other
Inherited Acquired Constitutional symptoms Rash Abdominal pain Diarrhea V/S Mass LAP Hepatosplenomegaly Arthritis Other WBC Hb Plt
HL
Hodgkin's Lymphoma
NHL
B Cell Lymphoma Precursor B-cell lymphoblastic leukemia/lymphoma
  • Precursor B-cell Lymphoma
Follicular lymphoma
  • Mature B-cell Lymphoma
  • Reciprocal translocation t(14;18)(q32;q21)
+ +
  • Painless swelling in the neck, axilla, groin, thorax and abdomen
  • Central and peripheral lymphadenopathy
+
Mantle cell lymphoma
  • Stage IV disease
  • B symptoms,
  • Generalized lymphadenopathy
  • Abdominal distention
  • Fatigue
  • Night sweats
  • Weight Loss
  • Extranodal involvement of gastrointtestinal (GI) tract, lungs, and central nervous system (CNS)
  • Generalized lymphadenopathy
  • Hepato-splenomegaly
  • Mental Retardation
  • Less commonly
    • Palpable masses in skin, breast, and salivary glands
CBC
  • Anemia and cytopenias are secondary to bone marrow infiltration
  • Lymphocytosis > 4000/µL
  • Elevated LDH
  • CD5+
  • B-cell antigen positive
    • CD19
    • CD20
    • CD22
  • Cyclin D1 is overexpressed.
  • Germinal centers filled by small-to-medium atypical lymphocytes.
  • Nodular appearance may be evident from expansion of the mantle zone in 30-50% of patients early in the disease.
Diffuse large B cell lymphoma
  • Mature B-cell Lymphoma
  • 8q24/MYC translocations
  • Hypermutated immunoglobulin genes
  • Mutation of BCL6, MYC, PAX5, PIM1, RhoH /TTFn, TP53 genes
  • Translocations involving c-MYC, BCL6, and IgH gene.
  • Fever
  • Night sweats
  • weight loss
Nl to ↓

Centroblastic

Immunoblastic

  • > 90% immunoblasts
  • Trapezoid shaped large lymphoid cells with significant basophilic cytoplasm

Anaplastic:

  • Very large cells with a round, oval, or polygonal shape that may resemble Reed-Sternberg cells
  • Hypergammaglobulinemia
Category Disease Etiology Inherited Acquired Demography History Constitutional symptoms Rash Abdominal pain Diarrhea V/S Mass LAP Hepatosplenomegaly Arthritis Other WBC Hb Plt PBS LFT PT/PTT Immunochemistry Histopathology Other lab findings Imaging Gold standard Associated findings
Burkitt lymphoma
  • Mature B-cell Lymphoma
  • Translocation of chromosome 8 myc locus such as:
    • t(8;14)
    • t(2;8)
    • t(8;22)
  • Medium-sized (~1.5-2x the size of a RBC) with uniform size ("monotonous") -- key feature (i.e. tumor nuclei size similar to that of histiocytes or endothelial cells)
  • Round nucleus
  • Small nucleoli
  • basophilic cytoplasm
  • Brisk mitotic rate and apoptotic activity
  • Cellular outline usually appears squared off
  • "Starry-sky pattern":
    • The stars in the pattern are tingible-body macrophages (macrophages containing apoptotic tumor cells)
    • The tumour cells are the sky
B cell chronic lymphocytic leukemia/small lymphocytic lymphoma
  • Mature B-cell Lymphoma
Marginal zone lymphoma Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type
  • Mature B-cell Lymphoma
Splenic marginal zone lymphoma
  • Clonal rearrangements of the immunoglobulin genes (heavy and light chains) .
    • Deletion 7q21-32
    • Translocations of the CDK6 gene located at 7q21.
  • B-cells replace the normal white pulp of the spleen.
  • The neoplastic cells compromise
  • Sinus invasion
  • Epithelial histocytes
  • Plasmacytic differentiation of neoplastic cells.
  • Splenic Hilar Lymph Nodes
  • Bone Marrow Biopsy
    • Splenic marginal zone lymphoma in bone marrow displays a nodular pattern with morphology similar to what is observed in the splenic hilar lymph nodes.
Nodal marginal zone B-cell lymphoma
  • Arise from memory B cells. Include
    • Splenic marginal zone lymphoma
    • Nodal marginal zone lymphoma
    • Extranodal marginal zone lymphoma.
  • Stimulation of antigen receptor by autoantigen and co-stimulatory molecule CD40.
  • Depends largely on its location
  • Gastric marginal zone lymphoma
    • Dyspepsia
    • Abdominal pain
    • Hemorrhage
  • Chronic infectious conditions or autoimmune processes, such as
    • H pylori gastritis
    • Hashimoto thyroiditis
    • Sjögren syndrome.
  • AE1/AE3
  • B-cell markers CD20, CD79a, CD10, CD23, and bcl-2 are expressed
  • Follicular cells in reactive zone
  • Centrocyte like cells in marginal zone lymphoma
Lymphoplasmacytic lymphoma (Waldenstrom’s macroglobulinemia)
  • Mature B-cell Lymphoma
Hairy cell leukemia
  • Production of cytokines, such as TNF alpha and IL-2R, provide important stimuli for malignant B cells proliferation in hairy cell leukemia.
    • The p38-MAPK-JNK cascade
    • The MEK-ERK cascade
    • The Phosphatidylinositol 3 kinase (PI3K)-AKT cascade
  • Tartrate-resistant acid phosphatase positive
  • CBC
  • Small cells with "fried egg"-like appearance
  • Well-demarcated thread-like cytoplasmic extensions
  • Clear cytoplasm
  • Central round nucleus
  • Peri-nuclear clearing ("water-clear rim" appearance)
Precursor T-cell lymphoblastic leukemia/lymphoma
  • Precursor T-cell Lymphomas


Adult T cell leukemia/lymphoma
T cell lymphoma Anaplastic large cell lymphoma
Cutaneous T-cell lymphoma Mycosis fungoides / Sézary syndrome
  • Cutaneous manifestaions
Peripheral T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma
Hepatosplenic gamma-delta T-cell lymphoma
Enteropathy-type intestinal T-cell lymphoma
Extranodal T-cell lymphoma, nasal type
Angioimmunoblastic T-cell lymphoma
Peripheral T-cell lymphoma, unspecified