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What condition:
Causes B cell production of excessive IgG (most common) or IgA, with decreased production of the other immunoglobulins
Serum protein electrophoresis by an "M" spike in the gamma globulin region
Bone marrow: Plasma cells >30%
Marked rouleaux
Increased ESR
Plasma cells and lymphs on blood smear
Bence Jones proteins (free light chains - kappa or lambda) found in urine
Causes: Multiple skeletal system tumors of plasma cells which cause lytic bone lesions and hypercalcemia, Increased blood viscosity due to excessive IgG or IgA, Prolonged bleeding, Kidney damage due to Bence Jones protein
Monoclonal gammopathy in Multiple Myeloma
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What condition:
Causes B cell production of excessive IgM (macroglobulin) and decreased production of the other immunoglobulins
Lymphadenopathy
Hepatosplenomegaly
Increased blood viscosity due to IgM production
Possible interference with platelet function, fibrin polymerization, and function of other coag proteins
Serum electrophoresis by an "M" spike in the gamma globulin region
Marked rouleaux
Increased ESR
Plasmacytoid lymphocytes
Plasma cells and Lymps on blood smear
Monoclonal gammopathy in Waldenstrom macroglobulinemia
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What condition:
Causes a proliferation of malignant cells in solid lymphatic tissue, lymphadenopathy, and intially localized but may spread to bone marrow and blood
Diagnosed: Tissue biopsy, CD surface markers, Cytogenetics, DNA analysis/PCR
Lymphoma
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What are the groups of lymphoma, according to WHO?
- Hodgkin B cell
- T/NK cell (non-Hodgkin) neoplasms
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What condition:
Has Reed-Sternberg (RS) cell found in lymph node biopsy are large, multi-nucleated cells each with prominent large nucleoli (B cell lineage)
Mild anemia
Eosinophilia
Monocytosis
Increased LAP score
Increase ESR during active disease state
Causes: Enlarged lymph nodes, Gastrointestinal tumors, B cell neoplasms (more common)
Hodgkin lymphoma
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What are the subtypes of Hodgkin lymphoma, according to WHO?
- Nodular sclerosis - 70% are this subtype; lowest EBV association
- Mixed cellularity - 20% are this subtype; highest EBV association
- Lymphocyte rich
- Lymphocyte depleted - uncommon
- All subtypes are associated with RS cells
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What does Mycosis funoides (cutaneous T cell lymphoma) cause?
- Cutaneous lymphoma causes skin itching, leading to ulcerative tumors
- Sezary syndrome: a variant of mycosis fungoides, presents as disseminated disease with widespread skin involvement and circulating lymphoma cells
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How does WHO classify Mycosis fungoides (cutaneous T cell lymphoma)?
T/NK cell neoplasm
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What cell markers are present in Mycosis fungoides / T/NK cell neoplasm?
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What condition:
Neutropenia
Anemia
Thrombocytopenia
Variable WBC count
Hypercellular marrow with bone marrow blasts >/=20% (WHO) or >30% (FAB)
Acute Myeloproliferative Disorder
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What FAB classification is consistent with the findings of:
Blasts exhibit myeloid markers CD13, CD33, CD34
Stain negatively with the usual cytochemical stains (MPO and Sudan black B (SBB))
FAB M0
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What FAB classification is consistent with the findings of:
90% or more marrow myeloblasts
May have Auer rods
SBB, MPO, and specific esterase positive
FAB M1
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What FAB classification is consistent with the findings of:
<90% marrow myeloblasts
May have Auer rods
Chromosome abnormality t(8;21)
SBB, MPO, and specific esterase positive
CD13, CD33 Positive
FAB M2 (AML with maturation)
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What disease is consistent with the findings:
>30% marrow promyelocytes with bundles of Auer rods (faggot cells)
Heavy azurophilic granulation
SBB, MPO, and specific esterase positive
CD13, CD33 positive
Chromosome abnormality t(15;17)
- Acute promyelocytic leukemia (APL)
- or FAB M3
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What disease is consistent with the findings:
>/=20% marrow myeloblasts (WHO) or >30% (FAB), may have auer rods
Proliferation of unipotential stem cell CFU-GM that gives rise to granulocytes and monocytes
Increased urine/serum lysozyme
SBB, MPO, specific and nonspecific esterase positive
CD13, CD33, and CD14 positive
- Acute myelomonocytic leukemia (AMML)
- or FAB M4
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What disease is consistent with the findings:
>/=20% or >30% marrow monoblasts
Nonspecific esterase positive
CD14 positive
- Acute monocytic leukemia (AMoL)
- or FAB M5
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What disease is consistent with the findings:
>/=20% or 30% marrow myeloblasts
>50% dysplastic marrow normoblasts
Malignant normoblasts are
- PAS positive
- CD45, CD71
Myeloblasts are
- SBB and MPO positive
- CD13, CD15, CD33
- Acute erythroleukemia (AEL)/Di Guglielmo syndrome
- or FAB M6
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What disease is consistent with the findings:
Proliferation of megakaryocytes and atypical megakaryocytes in the bone marrow
Blasts may have cytoplasmic blebs
Marrow aspiration is a dry tap
Blood shows pancytopenia
CD41, CD42, CD61
- Acute megakaryocytic leukemia (AMegL)
- or FAB M7
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What is the term for containing 2 cell populations that expresses
Myeloid antigens
Lymphoid antigens
Bilineage leukemias
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What is the term for occuring when myeloid and lymphoid antigens are expressed on the same cell?
Biphenotypic leukemia
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What oncogene is implicated in polycythemia vera, chronic idiopathic myelofibrosis, and essential thrombocythemia?
JAK2 oncogene
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What oncogene is associated in chronic myelogenous leukemia?
BCR/ABL
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What disease is consistent with the findings:
Proliferation of granulocytes
Increased M:E ratio
WBC between 50-500, with all stages of granulocyte production
Myelocytes dominate, may have few circulating blasts
LAP score is low
Chronic myelogenous leukemia (CML)
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What disease is consistent with the findings:
Chromosome translocation t(9;22)
All cell lines affected, except lymphs
Philadelphia chromosome
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What disease is consistent with the findings:
Proliferation of megakaryocytes
Platelets >1000
Platelet function abnormalities
Leukocytosis
Essential thrombocythemia (ET)
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What disease is consistent with the findings:
Malignant hyperplasia of mulipotential myeloid stem cell - causing an increase in all cell lines (polycythemia)
Erythrocytes most greatly increased despite decreased erythropoietin (EPO)
High blood viscosity
RBC in between 7-10
Hgb >20 g/dL
HCT >60%
Polycythemia vera (PV)
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What disease is consistent with the findings:
Myeloid stem cell disorder characterized by proliferation of erythroid, granulocytic, and megakaryocytic precursors in marrow with dyspoiesis
Progressive marrow fibrosis
Poikilocytosis with tear drop cells
Leukoerythroblastic anemia (immature neutrophils and NRBCs)
Abnormal morphology in all cell lines
Chronic idiopathic myelofibrosis
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What disease is consistent with the findings:
Anemia that is refractory (not responsive) to therapy
Oval macrocytes
Reticulocytopenia
dyserythropoiesis
Bone marrow blasts <5%
Peripheral blood blasts <1%
Refractory anemia (RA)
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