Neoplasitc Proliferative Disorders of Heme and Lymph
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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
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ALL | CHILDREN
lymphoblasts in blood and marrow
responsible to Tx
CD10
Thrombocytopenic bleeding
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AML | adults
myeloblasts and early promyelocytes
responds more poorly than ALL
AUER RODS
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Chronic Leukemias | more mature cells than acute leukemias
longer and better course
less responsive to Tx
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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
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Hairy Cell Leukemia | Hairy projections
TRAP + or tartarate-resistant acid phosphatase
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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
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General features of Myeloproliferative disorders | includes p.vera and chronic idiopathic myelofibrosis
Janus2 and JAK2V617F positive
proliferation of myeloid cells, increased serum uric acid
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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
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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
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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
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essential thrombocytopenia | thrombocytosis > 1,000,000
megakaryocytosis
bleeding and thrombosis
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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
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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
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Waldenstroms macroglobulinemia | M spike - IgM
Bence Jones proteins
no bone lesions
plasmacytoid lymphocytes
men > 50
hyperviscocity from IgM
retinal vascular dilation
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MGUS | M protein less than 2g/100mL
no Bence Jones proteins
less than 5% plasma proteins
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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
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Small Lymphocytic Lymphoma | B-cell lymphoma
indolent course
older
effacement of lymph node architecture
widespread nodal involvement
CLL - CD19, CD20 with CD5+ and CD10-
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Follicular Lymphoma | B-cell
indolent
older
MOST COMMON
angulated grooved cells
nodular lymph
t(14;18) bcl-2
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Mantle cell lymphoma | t(11;14)
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Extranodal marginal zone B-cell lymphoma of MALT type | Sjogren, Hashimoto, H. Pylori associated
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Burkitt Lymphoma | B-cell
EBV
starry sky
macrophages are stars
t(8;14)
c-myc
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cutaneous T-cell lymphomas | 1) mycosis fungoides - erythema, eczema, psoriasis, CD4+ with cerebriform nucs in epidermis
2) sezary syndrome - skin lesions and cerebriform nuclei
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Created by:
burkill
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