Hodgkins and Non-Hodgkins Lymphome

Question 1

list the non-Hodgkins Lymphomas

Answer 1

Acute Lymphoblastic Leukemia
Mantle Cell Lymphoma
Follicular Lymphoma
Burkitt Lymphoma

Question 2

Mantle cell lymphoma

Answer 2

B-cell neoplasm composed of monomorphic lymphocites w/ irregular nuclei

Express B-cell markers CD19, CD20, CD5
Express Cyclin1D+, CD1+, and BCL6+

BCL1 gene rearrangement at 11q13, constant overexpression of cyclin D1 t(11;14)(q13;32) involving BCL1 gene + IGH gene

Clinical features: Avg age of diagnosis: 60 yrs, M:F = 2:1. Localized mostly in lymph nodes. Pts present at stage 3-4 w/ lymphadenopathy, hepatosplenomegaly.

Hyalinized small vessels often present.

Moderately aggressive

Question 3

Follicular lymphoma

Answer 3

Germinal center B-cell lymphoma

40% of adult lymphomas, age of onset 60 yrs. Pts mostly asymptomatic. Some have spleen enlargement, Waldeyer’s ring, GI tract, skin, and soft tissue involvement

Poorly defined mantle zone, lack of polarization. Have centrocytes and centroblasts.

Immunophenotype: CD19+, CD20+, BCL2+

Germinal center B-cells = CD10+ and BCL6+

Cytogenetics: t(14;18)(q32;q21) Placed BCL2 under promoter. Overexpression induces hyperplacia.

Question 4

Burkitt Lymphoma

Answer 4

germinal center B-cell lymphoma

Highly aggressive, presents in extranodal sites or in leukemic form. Monomorphic medium sized B-cells w/ basophilic cytoplasm and high mitotic rt.

Clinical features: typical in malaria belt, 4-7 yrs of age, sporadic in children and young adults. Found in HIV pts as an immunodeficiency associated lymphoma.

Often found in jaw and facial bones (50%) others in distal ileum, cecum and omentum

Immunophenotype: CD19+, CD20+, high proliferation index (nearly 100% w/ Ki-67 staining,) EBV mostly positive

Cytogenetic findings: t(8;14)(q24;32) MYC gene next to IGH

VERY AGGRESSIVE but potentially cureable

Question 5

CLL/SLL

Answer 5

CLL:mature lymphocytosis of > 5 x10^9/L, sustained over 3 months.

Monoclonal antibodies w/ mature phenotype. CD5+, weak CD22, weak CD11c

SLL: same thing but disease predominated is extramedullary sites.

Median age 65 yrs, M:F ratio 2:1. Most are aymptomatic.

Common genetic findings: 
Deletion of 13q14 (common + favorable)
Trisomy 12
Deletion of 11q22-23
Deletion of 17p13 (adverse prognosis) 

Prognosis:
Favorable: CD38-, ZAP70-, germline IGH@V, pre-germinal center
Unfavorable: opposite

Question 6

List plasma cell myeloma subtypes

Answer 6

Multiple Myeloma
Monoclonal Gammopathy of Undermined Significance
Solitary plasmacytoma of bone
Extraosseos Plasmacytoma

Question 7

Multiple Myeloma

Answer 7

bone marrow based, M-protein found in serum or urine

Diagnostic criteria: M-protein in serum or urine. Related organ or tissue impairment. Hypercalcermia, renal insufficiency, anemia, bone lesions (CRAB)

Pts=50yrs old. Bone pain most common symptom.

IgM M protein found in 55% of pts, anemia in 2/3 of patients

Blood smear: rouleax formation

Question 8

Monoclonal Gammopathy of Undetermined Significance

Answer 8

Monoclonal immunoglobulin in serum or urine w/ no evidence of plasma cell myeloma

Probs a precursor to multiple myeloma, transformation 1.5% per year

Question 9

Solitary Plasmacytoma of Bone

Answer 9

Localized tumor of the bone made of cells of multiple myeloma

Single bone lesion w/ monoclonal plasma cells

Question 10

Extraosseos Plasmacytoma

Answer 10

localized plasma cell tumors that arise in tissues outside bone marrow.

Patients: 55 yrs, 2/3 are male

Usually occur in respiratory tract, nasal passages, sinuses, oropharynx and larynx

Question 11

List the major subtypes of classical Hodgkin Lymphoma

Answer 11

Nodular Sclerosis Classical - most frequent, found in young adults, predominantly females. Occurs above diaphragm. Thickened lymph node capsule+lacunar cells

Lymphocite-Rich Classical - about 5% of CHLs. Nodular growth pattern, residual germinal centers.

Mixed Cellularity Classical - 20-25% of HL, children and older patients. Lack broad band of collagen.

Lymphocyte-depleted Classical - least frequent type. EBV+, aplastic and bizarre. A CRAPTON of Reed-Sternberg Cells

Question 12

List and describe the two subtypes of Hodgkins Lymphoma

Answer 12

Nodular lymphocyte predominant subtype (NLPHL) - indolent malignancy, popcorn or lymphocyte predominant cell

CHL (Classical Hodgkins Lymphoma) - malignant cells are a minority. Reed-Sternberg Cells in appropriate inflammatory background. CD30+ and CD15+