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18 Cards in this Set

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Hairy cell Leukemia
B-cell- Chronic Leukemia
Key words- middle aged white males, splenomegaly, pancytopenia, infection
high remission rate and indolent course
Express- B-cell CD 19 and 20 monocyte-associated antigens CD11c, CD25, and CD103
Essential Thrombocytosis
abnormally large and atypical platelets (including megakaryocytes)
Older pts > 60- 12-15 year survival
can cause thrombosis or bleeding
Myleodysplastic Syndromes
Precursors to AML- often a result of prior radiation and chemo
1. Refractory Anemia
2. RA with Excess Blasts
3. RAEB in transformation
4. RA with Ringed Sideroblasts
5. CMML- chronic monomyelocytic leukemia
Myeloproliferative Syndromes
1. Polycythemia vera (RBC)
2. CML- Chronic myelocytic leukemia (WBC)
3. Essential thrombocytosis(Platelets- proliferation of platelets and megakaryocytes)
4. MMMF- idiopathic myelofibrosis (Stroma- hypocellular marrow)
AML (Acute Myelogenous Leukemia)
M1-without differentiation
M2-with maturation (Auer Rods)
M3-Promyelocytic (Auer Rods and t15:17)
M4-myelomonocytic
M5-monocytic (tissue infiltration)
M6-erythroleukemia
M7-megakaryocytic
Acute Leukemia Presentation
1. WBC= >100,000 (spilling into the blood) or <1000(too packed in the marrow to get out)
2. Mediastinal Mass
3. Skin, Testes, gingiva, meninges
4. Pancytopenia (or 2+ cytopenias)
Philidelphia Chromosome
t(9:22) BCR-ABL- tyrosine kinase
CML (if seen in AML it means they had CML and it converted)
ALL- bad prognostic factor
ALL (Acute Lymphocytic Leukemia) prognostic factors
*Disease of kids
Good= age 1-9, Hyperploidy
Bad= high WBC count >50,000, Philidelphia chromosome, infant or adolecent/adult (T cell), low platelets and Hb
Types of NH Lymphoma
1. Precursor B cell- B-ALL kids with pancytopenia (TdT+, CD 10, 19, 20+ no Ig genes, starry sky, t(9:22))
2. Precursor T cell- T-ALL teenage males with mediastinal mass (TdT+, CD 2, 5, 7+, starry night-can't tell apart from preB, TCR translocation)
3. CLL most common WW (mature cells- only CD19, 20+ with Ig genes, smudge cells)
4. SLL is CLL confined to lymph nodes
5. Follicular Lymphoma- most common in US (t(14:18) IgH BCL2, slow growing-anti-apoptotic, incurable
6. Diffuse large B cell Lymphoma- Primary Effusion= AIDS associated (EBV or HHV8) (very aggressive)
7. Burkitt Lymphoma- rarely leukemic (t(8:14)Myc/IgH (2:8)(8:22), African/Endemic/AIDS= EBV see in jaw, sporadic= Abdomen, starry sky marcophages)
8. Multiple Myeloma- (mature plasma cells, Bence Jones, lytic lesions, very aggressive)
Hodgkin Lymphoma
young adults
Staging is important (spread through contiguous nodes)
Reed-Sternberg cells
3 Types: 1)nodular sclerosis (Lucunar cells, collagen banding, most common) 2)mixed 3)lymphocitic
CML
Chronic Myeloid Leukemia
Philadelphia Chromosome t(9:22) BCR-ABL
Granulocytic precursors- see marked leukocytosis, with a predominance of neutrophils, metamyelocytes, and myelocytes
Multiple Myeloma
mature plasma B cell lymphoma
adults age 50-60 (AA more than whites)
Labs= >30% plasma cells in bone marrow, monoclonal IgG in serum/urine
Complications=
1.Lytic lessions-hypercalcemia and fractures
2. monoclonal IgG, Bence-Jones protein, myeloma kidney, infection
3. amyloidosis
Flow Cytometry for Lymphoma
Front Scatter= cell size
Side Scatter= cell complexity (maturity)
Mediastinal mass differential
teratoma
lymphoma
thymoma
thyroid carcinoma
Normal Splenic Architecture
White Pulp= T-cells (around central arteries) and B-cell (off to the side)
As the cells go through the splenic artery, they reach the sheathed capillaries (porous and surrounded by a sheath of macrophages), where they can move into the interstitium of the spleen (choice E is correct). Then in extra-capillary space before moving into the splenic vein and exit the spleen (unless they are engulfed by APCs first!).
red pulp= many RBCs exiting into the splenic interstitium
Normal Lymph Node Architecture
connective tissue capsule surrounding the node

Directly below the capsule is the marginal sinus, where lymph flows in and the filterable substance is distributed all around the lymph node

lymphoid follicles= B-cells -two types of follicles:
Primary follicles – no visible central clearing, these follicles= inactive, Secondary follicles – reactive follicles-germinal centers (relative clearing) where the B-cells actively proliferate

Between the follicles lie the paracortical areas= T-cells

medullary cords (lighter staining)= macrophages and plasma cells

medullary sinus= efferent lymph vessels leave the node
Generalized Lymphadenopathy Ddx
lymphoid chains around the body
1. Systemic infections – viral (HIV, EBV, CMV) (T cells in LN), bacterial (B cells in LN), mycobacterial, fungal, protozoal
2. Inflammatory disease – lupus, rheumatoid arthritis, sarcoidosis
3. Hematopoietic malignancies – Hodgkin’s disease, non-Hodgkin’s lymphoma (NHL), leukemia
4. Drug reactions – dilantin
Localized Lymphadenopathy Ddx
local only
1. Localized infections – nodes draining pyogenic infections: oral (pharyngitis), otitis media; TB; cat-scratch disease (Bartonella)
b) Neoplastic – solid tumors that have metastasized