Heme Malignancies

Question 1

Paracortex

Answer 1

mixed lymphocytes, histiocytes, macrophages, Langerhans cells, interdigitaing dendritic cells, high endothelial venules

Question 2

Structures of Lymph node:

Answer 2

-capsule [thin, fibrous]
-cortex
.lymphoid follicles [1/2 mostly B cells, mantle (outer- all B cells), germinal center (inner. light zone- centrocytes, Tcells, macrophages, others. dark zone- centroblasts)]
.paracortex [mixed cell population- mostly T cells (moth-eaten appearance)]
-medulla [medullary cord, sinus]

Question 3

WHO general classification of lymphoid neoplasms (5)

Answer 3

• microscopic appearance (of malignant cells)
• histologic growth patterns (of malignant cells in tissue)
• presence/absence of cytogenetic findings
• relative amount of malignant cells present in blood/marrow
• presence/absence of certain cell surface markers

Question 4

*CLL (Chronic Lymphocytic Leukemia)/ SLL (small Lymphocytic Lymphoma):

Answer 4

• most common Leukemia
• naive B cells co-expressing CD5, CD20
• ↑Lymphocytes, ↑smudge cells
• involvement of lymph nodes, lymphadenopathy
  complications: Hypogammaglobulinemia [infection is most common cause of death in CLL/SLL], AIHA, Richter transformation [morphs into Diffuse Large B-Cell Lymphoma]- marked by enlarging nodes/spleen
• CLL: >5*10^9/L abs mature lymphocytosis, 3+months.
• SLL: predominantly extramedullary involvement
• CD5/19/23/20+
• CD10-, FMC7-

-del 13q14, most common genetic alteration (>50%) of CLL, associated with favorable prognosis

Question 5

*Follicular Lymphoma:

Answer 5

-germinal center B-Cells (centrocytes & centroblasts)
-CD10/19/20+, BCL2/6+
-CD5/23-
.bcl2= suppresses apoptosis, normally down-regulated (normal B-cells are negative for bcl2)
-40% of US, 20% world lymphomas
-60yo, M=F
-often asymptomatic except for lymphadenopathy
-DDX.. disrupts normal lymph node architeture, lack of tingible body macrophages in germinal centers, monoclonal

-t(14;18)(q32;q21)

Question 6

*Mantle Cell Lymphoma:

Answer 6

• B-calls w irregular nuclei
• CD5/19/20+, BCL1+
• 3-10% NHL
• 60yo, M=F
• scattered epithelioid histiocytes with ‘starry sky’ appearance

-t(11;14)(q13;q32)

Question 7

Lymphadenopathy:

• define
• painful
• painless

Answer 7

• LAD. abnormality in size (enlargement), quantity (increase), or consistency of lymph nodes
• acute infections (draining a region)
• chronic inflammation, metastatic carcinoma, lymphoma

Question 8

Indolent Lymphomas(2):
-define

Answer 8

-relatively good prognosis, mostly nodular/follicular morphology, usually not curable/relapse in late stages

• CLL/SLL
• Follicular Lymphoma

Question 9

Aggressive Lymphomas(3):
-define

Answer 9

-grows, spreads quickly with severe symptoms, more likely to cure

• Mantle Cell Lymphoma
• Burkitt’s Lymphoma
• DLCBCL (Diffuse Large B-Cell Lymphoma)

Question 10

*Burkitt’s Lymphoma

3 subtypes

Answer 10

Endemic: equatorial Africa, 4-7yo, jaw/abdomen, 95% assoc. w EBV

Sporadic: kids-young adults, ileocecal area, ~30% assoc. w EBV

Immunodeficieny-assoc. BL:
mostly in HIV pts, 25-40% assoc. w EBV

• monomorphic medium sized cells w ‘starry sky’ pattern, ‘squared off’ borders, round nuclei w up to several small nucleoli, deeply basophilic cytoplasm w lipid vacuoles
• CD19/20/10+, BCL6+, MYC+

Question 11

Cell markers (4)

Answer 11

• CD20 [BC specific. + in mantle, germinal center BCs. CD19,22 also BC markers]
• CD3 [TC specific. + in paracorical TCs. CD4,5,8 also TC markers]
• BCL6 [specific in normal germinal BCs]
• CD10 [specific in derived lymphomas]

Question 12

Lymphomas by location (5)

Answer 12

Marrow: B-ALL/LBL (B-cell acute lymphoblastic leukemia/ lymphoblastic lymphoma)

Mantle: Mantle Cell Lymphoma

Germinal Center (B-cell):
Follicular lymphoma, Burkitt Lymphoma, Hodgkin Lymphoma

Pre/Post germinal Center (B-Cell): CLL/SLL

Plasma Cell: Plasma Cell myeloma (multiple myeloma)