WBC Disorders (Sloka) Flashcards
Name the WHO classification system of lymphoid neoplasms.
- Precursor B cells Neoplasms
- Peripheral B cell neoplasms
- Precursor T cell neoplasms
- Peripheral T cell and NK neoplasms
- Hodgkin’s Lymphoma
Know the markers (CD10 for example) by using Dillon’s quizlet!
pryo6619
MC cancer and leukemia in children less than 15 y/o. Peak age is 3 y/o
ALL (B-ALL)
Neoplastic accumulation of lymphoblasts (> 20%) in the bone marrow
Lymphoblasts are characterized by positive nuclear staining for TdT, a DNA polymerase.
TdT is absent in myeloid blasts and mature lymphocytes.
ALL
Characteristics of B-ALL
CALLA: CD10
TdT+
T(12,21) or philadelphia chromosome (9,22)
Hyperdiploidy (SS)
“Hyper kids like playing w/B-ALLS”
Prognosis of B-ALL
95% complete remission
75-85% cures
Lab findings of B-ALL?
WBC elevated but large range. Can be normal initially.
NN anemia!!!!
Thrombocytopenia!!!! (all acute leukemia’s have both)
Lymphoblasts- greater than 30% in BM (acute)
CD10 neg but TdT pos
Anterior mediastinal mass/acute leukemia found in an adolescent male.
Likely dx?
T-ALL!
MC leukemia of all?
CLL
Disorder:
Neoplastic proliferation of Bcells (CD20+); associated with EBV
Burkitt’s Lyphoma
MC leukemia of patients older than 60?
CLL
Burkitt Lymphoma is driven by translocations of _____. t(&)
C-myc. t(8,14)
Starry sky is caused by and is associated with Burkitt’s.
clear spaces with macrophages containing debris (“tingible” body macrophages)
MCC NHL?
Diffuse large B cell lymphomas
MCC of generalized lymphadenopathy?
SS
CLL
In CLL, lymphoblast count in BM?
How about:
anemia
thrombocytopenia
what characteristic cell will you see in CLL?
MCC of death in CLL?
WBC count varies “Smudge” cells NN anemia 50% Thrombocytopenia 40% Hypogammaglobulinemia- MCC of death due to infections. BM -
Disorder?
BCL2 on chromosome 18 translocates to the Ig heavy chain locus on chromosome 14.
Results in over-expression of Bcl2, which inhibits apoptosis
Follicular Lymphoma
Neoplastic proliferation of mature B-cells.
Middle-aged men B cell TRAP, BRAF Splenomegaly 90%, lymphadenopathy is rare Pancytopenia (especially monocytopenia)
Hairy Cell Leukemia
Pancytopenia is in the peripheral blood.
Increased lymphocytes and smudge cells are seen on blood smear
CLL
MCC of death in CLL?
Infections due to Hypogammaglobulinemia
Disorder?
Neoplastic proliferation of small B cells (CD20+) that form follicle-like nodules?
Follicular Lymphoma
Neoplastic proliferation of small B-cells.
Associated with chronic inflammatory states such as Hashimoto thyroiditis
Sjogren syndrome
H pylori gastritis
if in mucosal sites, called MALToma
Marginal zone lymphoma
Patient comes in with: WBC elevated NN anemia Lymphoblasts greater than 30% in BM Thrombocytopenia
Likely dx?
Leukemia
Acute.
Lymphoblastic
Patient does not have lympadenopathy, has splenomegaly, and is pancytopenic in peripheral blood.
Trap +
Most likely dx?
Hairy cell leukemia
Neoplastic proliferation of naive B cells that co-express CDS and CD20
CLL
Neoplastic proliferation of small B cells (CD20+).
Clinically presents in late adulthood with painless lymphadenopathy.
Driven by t(11;14)
Cyclin Dl gene on chromosome ll translocates to Ig heavy chain locus on chromosome 14.
Over expression of cyclin D1 promotes Gl/S transition in the cell cycle, facilitating neoplastic proliferation.
Mantle Cell Lymphoma
SS.
Disorder?
Often develop in patients with abnormal immune states (e.g. SLE, Sjogren’s’ syndrome, renal transplantation)
May occur at any age
Usually elderly adults
Diffuse Large B cell lymphoma
SS.
The MC form indolent lymphoma?
Follicular lymphoma
SS:
Most common monoclonal Plasma Cell Dyscrasia?
Monoclonal gammopathy of undetermined significance (MGUS)
What is multiple myeloma?
Malignant proliferation of plasma cells in the bone marrow. One plasma cell clone is proliferated while the others are suppressed leading to monoclonality.
Plasma Cell Dyscrasia:
Most commonly due to an increase in IgG
Other plasma cell clones are suppressed
Multiple Myeloma
Describe Lymphoplasmacytic lymphoma (Waldenstrom’s macroglobulinemia)
B-cell lymphoma with monoclonal IgM production.
Plasma Cell Dyscrasia:
Small IgG M spike in elderly patients
Plasma cells
Monoclonal gammopathy of undetermined significance (MGUS)
Characteristics of multiple myeloma?
High IgM
Bone pain with hypercalcemia
lytic bone lesions
Elevated serum protein –> Rouleaux formation
Bence Jones protein (free light chain excreted in urine; myeloma kidney: Renal failure 30-50%)
Amyloidosis- free light chains circulate in serum and deposit in tissues
Monoclonal antibody
NO generalized lymphadenopathy! (compare to Waldenstrom’s)
MCC of death in multiple myeloma?
infection due to monoclonal antibody
M spike with IgM
BJ protein is present
Generalized lymphadenopathy
Anemia and bone marrow but no lytic lesions
liver, and spleen involvement
Hyperviscosity syndrome due to increased IgM:
retinalhemorrhages, strokes, platelet aggregation defects
Lymphoplasmacytic lymphoma (Waldenstrom’s macroglobulinemia)
Which disorder?
Epidemiology MC in blacks Rare under age 40 Peak incidence at age 50-60 Increased risk with radiation exposure M-spike in 80-90%
Multiple Myeloma