Heme II Flashcards
What comprises the lymph node parenchyma?
Capsule
Cortex
Medulla
What lymph node sinuses are there?
Subcapsular
Cortical
Medullary
What is CD20? Where is it positive
- B-cell specific marker
- Positive in mantle zone cells, germinal center B-cells
- Other B-cell markers: CD19, CD22
What is CD3? Where is it positive?
-T-cell specific marker
Positive in paracortical T-cells
Other T-cell markers: CD4, CD5, CD8
What is the difference between Chronic Lymphocytic Leukemia / Small Lymphocytic Lymphoma (CLL/SLL)?
CLL: peripheral blood lymphocytosis -monoclonal mature lymphocytes
- **most common leukemia in the Western world and ~ 30% of all leukemia
- elderly patients usually asymptomatic whereas other patients may have fatigue, anemia, etc.
SLL: extramedullary involvement; similar morphology and immunophenotype to CLL
-accounts for ~7% of Non-Hodgkin lymphoma
Median 65 years, male predominance, M>F
What do Chronic Lymphocytic Cells look like in the periphereal blood?
Lymphocytosis!!!
Typical CLL cells: Size: small and monotonous Nuclei: round, condensed chromatin Nucleoli: inconspicuous Cytoplasm: scant and agranular Frequent smudge cells and basket cells Prolymphocyte (arrow): variable Size: larger Nuclei: round, more fine chromatin Nucleoli: prominent Cytoplasm: abundant
What do the nodes look like in CLL or SLL?
- loss of nodal architecture with diffuse infiltration
- Proliferation centers: pale areas containing transformed larger cells
What do tumor cells look like for CLL/SLL?
-small in size and have round nuclei with clumped chromatin
What is follicular lymphoma?
-A lymphoma of germinal center B cell origin
-40% of adult lymphomas in US, 20% worldwide
-Age: mostly adults, median 60 years
-Gender: male = female
Locations: mostly lymph nodes, also spleen, bone marrow, Waldeyer’s ring, GI tract, skin and soft tissue
-Clinical presentations:usually at stage III or IV at diagnosis, some have bone marrow involvement; however, patients often asymptomatic except for lymphadenopathy
What is mantle cell lymphoma? How does it compare with other small B-cell lymphomas?
- 3-10% of NHL
- Median age 60 years, M:F=2:1
- Location: mostly lymph nodes, also spleen and bone marrow; most common extranodal sites, gastrointestinal tract and Waldeyer’s ring
- Clinical presentations: most patients present with stage III or IV with lymphadenopathy, hepatosplenomegaly; >50% of cases with massive splenomegaly and marrow involvement
- In contrast to other small B-cell lymphoma that are mostly indolent, MCL is usually moderately aggressive
What is the Immunophenotype of Mantle Cell Lymphoma?
- Positive for cyclin D1 (BCL1), which is negative in most of other B-cell lymphomas
- translocation from ch. 14 to ch. 11 which induces persistent overexpression of BCL1 protein, which accelerates passage through the G1 phase and increases risk of lymphoma development.
What is Burkitt’s lymphoma? What is the difference for endemic vs. sporadic vs immunodeficiency associated?
Endemic BL:
- malaria belt of equatorial Africa
- Age: peak 4-7 years of age
- Locations: jaw or abdomen
- 95% associated with Epstein-Barr virus infection (EBV)
Sporadic BL:
- Mostly in children or young adults
- Common location: ileocecal area
- ~30% associated with EBV infection
Immunodeficiency-associated BL:
Primarily in HIV patients
-25-40% associated with EBV infection
***ALL THREE ASSOCIATED with EBV
What is the typical chromosome issue for Burkitt’s Lymphoma?
t(8;14)(q24;q32)
–>This translocation fuses the MYC gene at 8q24 next to the IGH locus at 14q32, resulting in overexpression of the transcription factor MYC.
What is the prognosis of Burkitt’s lymphoma?
- Highly aggressive but potentially curable
- Curable with aggressive therapy in ~60% cases
What is the definition of a plasma cell neoplasm,? Where do most plasma cell neoplasms originate?
- clonal proliferation of plasma cells that secrete a SINGLE CLASS of immunoglobulin or a polypeptide subunit of a single immunoglobulin, which is usually detectable as a monoclonal protein (M protein) on serum or urine protein electrophoresis.
- Most plasma cell neoplasms originate as bone marrow tumors, occasionally also extramedullary sites.
What are the WHO classifications of plasma neoplasms?
-Monoclonal gammopathy of undetermined significance (MGUS)
-Plasma cell myeloma (Asymptomatic (smoldering) myeloma, Nonsecretory myeloma, Plasma cell leukemia)
-Plasmacytoma (Solitary plasmacytoma of bone, Extraosseous (extramedullary) plasmacytoma)
-Immunoglobulin deposition diseases(Primary amyloidosis,
Systemic light- and heavy-chain deposition diseases)
-Osteosclerotic myeloma (POEMS syndrome)
What is the definition of a plasma cell myeloma? How is the diagnosis made
- bone marrow–based, multifocal plasma cell neoplasm associated with an M protein in serum or urine.
- originates from the marrow with disseminated marrow involvement in most cases. Other organs may be secondarily involved.
- The diagnosis made by a combination of clinical, morphologic, immunologic, and radiographic information. -spectrum from asymptomatic to highly aggressive
What is the diagnostic criteria for symptomatic plasma cell myeloma (PCM)?
- M protein in serum or urine: no minimal level of M protein, but in most cases it is >30g/L of IgG, >25g/L of IgA, or >1g/24 hours of urine light chain
- Bone marrow clonal plasma cells or plasmacytoma: no minimal level, but in most cases the monoclonal plasma cells usually >10% of nucleated cells in the marrow.
- Related organ or tissue impairment: hypercalcemia, renal insufficiency, anemia, bone lesions (CRAB).
What are the clinical features of plasma cell myeloma (PCM)?
- Incidence: ~ 4/100,000 persons per year in US
- Age: median ~ 68 years, rare in adults 50 years of age
- Most frequent symptom at presentation: bone pain in the back or extremities due to lytic lesions or osteoporosis. -Weakness and tiredness, often related to anemia
What are the radiological findings of plasma cell myeloma?
- Lytic bone lesions, osteoporosis or fractures in 70-85% of myeloma patients at diagnosis
- Locations: vertebra, pelvis, skull, rib, femur, and proximal humerus
What are the lab findings of plasma cell myeloma?
- Serum/urine protein electrophoresis: “gold standard” for characterizing heavy and light chains and detecting M protein, plasmacytoma, heavy-chain disease, and light-chain deposition disease and following treatment of myeloma
- M proteins: IgG ~ 55% of cases, IgA ~20%, light chain only 20%, IgD/IgE/IgM/biclonal myeloma all rare,
What are the peripheral blood findings of plasma cell myeloma?
- Plasma cells are found on blood smears in ~15% of cases, usually in small numbers
- Rouleaux formation is often present when the M protein is markedly elevated
Is bone marrow biopsy needed to confirm a myeloma diagnosis?
Yes, nearly always required to evaluate the cytology of tumor cells and degree of marrow involvement, and to obtain specimen for necessary genetic/molecular studies.
What is Monoclonal Gammopathy of Undetermined Significance (MGUS) generally?
-monoclonal immunoglobulin in the serum or urine of a patient with NO evidence of plasma cell myeloma, amyloidosis, Waldenström’s macroglobulinemia, or other lymphoproliferative disorder or disease known to produce monoclonal immunoglobulins.
