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Burkill-NeoHemo
Neoplasitc Proliferative Disorders of Heme and Lymph
Question | Answer |
---|---|
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 |