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Neoplasitc Proliferative Disorders of Heme and Lymph

Common associations with Acute Leukemias Blasts in the bone marrow and peripheral blood Children and >60 Short path marked by anemia, infection, hemorrhage and death in 6 months
ALL CHILDREN lymphoblasts in blood and marrow responsible to Tx CD10 Thrombocytopenic bleeding
AML adults myeloblasts and early promyelocytes responds more poorly than ALL AUER RODS
Chronic Leukemias more mature cells than acute leukemias longer and better course less responsive to Tx
CLL neoplastic lymphoid cells - B-cells No plasma cells - cannot differentiate >60 yo B-cells CD19, CD20, also CD5+ and CD10- SMUDGE CELLS peripheral WBC 50-200 Complications: Warm antibody autoimmune hemolytic anemia, hypogammaglobulinemia and infectio
Hairy Cell Leukemia Hairy projections TRAP + or tartarate-resistant acid phosphatase
CML 35-50 year olds Philadelphia chromosome t(9;22) Bcr-abl codes for tyrosine kinase protein leukocytosis WBC 50-200 mid-late myeloid precursor - segmented forms reduced leukocyte alkaline phosphatase (LAP) score
General features of Myeloproliferative disorders includes p.vera and chronic idiopathic myelofibrosis Janus2 and JAK2V617F positive proliferation of myeloid cells, increased serum uric acid
Polycythemia Vera - abnormal serum RBC levels with decreased EPO and normal O2 erythrocytosis increasing granulocytes and platelets splenomegaly thrombotic or hemorrhagic phenomena decreased EPO pruritis
Secondary causes of polycythemia chronic hypoxia from pulm diseases, high altitiude, heavy smoking inappropriate production of EPO from renal, hepatocellular, or cerebellar endocrine abnormalities like a pheochromocytoma ro Cushings
Chronic Idiopathic hyelofibrosis or agnogenic myeloid metaplasia and myelofibrosis with myeloid metaplasia extramedullary hematopoesis because the marrow has become fibrotic TEAR DROP CELLS anemia splenomegaly
essential thrombocytopenia thrombocytosis > 1,000,000 megakaryocytosis bleeding and thrombosis
Infectious Mononucleosis non-neoplastic lymphoid proliferation EBV is cause B-cell affinity Young adults atypical lymphocytes (CD8+ T cells) marked by antibodie: anti-EBV, heterophil (sheep erythrocyte attackers) Sx: sore throat, fever, general lymphadenopathy spleen can r
Multiple Myeloma plasma cell disorder - 40-50 yo lytic bone lesions punched out lesions OAF secreted by plasma cells bone pain M protein - IgG or IgA of kappa or lambda Hyperglobulinemia Bence Jones proteins rouleaux infections increase Hyper Ca amyloid myelom
Waldenstroms macroglobulinemia M spike - IgM Bence Jones proteins no bone lesions plasmacytoid lymphocytes men > 50 hyperviscocity from IgM retinal vascular dilation
MGUS M protein less than 2g/100mL no Bence Jones proteins less than 5% plasma proteins
Hodgkin lymphoma malignant Young adults B-symptoms Reed-Sternberg Lymphocyte depletion = many RS cells = bad Lymphocyte rich = EBV Lymphocyte predominance = good prognosis, few RS cells Nodular Sclerosing - women, fibrous bands, lacunar cells, good prognosis
Small Lymphocytic Lymphoma B-cell lymphoma indolent course older effacement of lymph node architecture widespread nodal involvement CLL - CD19, CD20 with CD5+ and CD10-
Follicular Lymphoma B-cell indolent older MOST COMMON angulated grooved cells nodular lymph t(14;18) bcl-2
Mantle cell lymphoma t(11;14)
Extranodal marginal zone B-cell lymphoma of MALT type Sjogren, Hashimoto, H. Pylori associated
Burkitt Lymphoma B-cell EBV starry sky macrophages are stars t(8;14) c-myc
cutaneous T-cell lymphomas 1) mycosis fungoides - erythema, eczema, psoriasis, CD4+ with cerebriform nucs in epidermis 2) sezary syndrome - skin lesions and cerebriform nuclei
Created by: burkill