Hematology WBC disorders Flashcards
CD34+
Hematopoetic Stem Cells
GM-CSF & G-CSF treat?
Neutropenia
Decreased CD16
CD16= Fc rec
Immature Neutrophils
WBC increased by Type I hypersensitivity, Hodgkin’s lymphoma
Eosinophils
WBC increased by CML
Basophils
WBC increased by Bordatella pertussis
Lymphocytes
Can’t leave circulation
CD8+ Tcell leukocytosis
EBV
sometimes CMV
TdT+ stain
Down Syndrome after 5
ALL
Acute Lymphoblastic Leukemia
CD10, CD19, CD20
B-ALL
MC vs. T-ALL
TdT+
t(12:21)
B-ALL
MC in kids, good prognosis
t(9:22) philadelphia
TdT+
CD2-CD8
Negative CD10
T-ALL
TdT+
MPO+
Acute Myeloid Leukemia AML
Auer rods
t(15:17)
Acute Promyelotic Leukemia
RAR (17) -> blocks maturation -> promyelocyte^ -> DIC risk^
Infiltrate gums
Acute Monocytic leukemia
Lacks MPO
AML subtype
Down Syndrome before age 5
Acute megakaryoblastic leukemia
Lack MPO
AML subtype
CD5 and CD20
MC leukemia overall
Chronic Lymphocytic Leukemia Smudge cells Hypogammaglobulinemia Infection MCC of death Autoimmune hemolytic anemia Transformation (Richter) to Diffuse Large B-cell Lymphoma
TRAP+
Hairy Cell Leukemia
Lacks LAD
Splenomegaly (Red Pulp)
Hairy Cell Leukemia
Dry tap on BM aspiration due to fibrosis.
Lacks LAD
Responds to 2-CDA
Hairy Cell Leukemia
(cladribine) Adenosine deaminase inhibitor - causes adenosine to accumulate and becomes toxic in neoplastic B-cells
Associated with HTLV-1
Adult T-Cell Leukemia/lymphoma (ATLL)
MC seen in Japan and Caribbean
Rash, Generalized LAD with Hepatosplemnomegaly, and lytic (punched-out) bone lesions with hypercalcemia
Adult T-Cell Leukemia/Lymphoma (ATLL)
Neoplastic proliferation of mature CD4+ T cells
Adult T-Cell Leukemia/Lymphoma (ATLL)
Mycosis Fungoides
Skin rash, plaques, and nodules created by neoplastic cells
-Pautrier Microabscesses
Mycosis Fungoides
Neoplastic proliferation of mature CD4+ T cells that infiltrate the skin
Sezary syndrome
Mycosis Fungoides
Cells spread to involve blood.
Characteristic lymphoctytes with cerebriform nuclei on blood smear
Neoplastic proliferation with average age of 50-60 years
Myeloproliferative disorders
CML, PV, ET, Myelofibrosis
Hypercellular BM
Increased risk for hyperuricemia and gout
Progression to BM fibrosis or transformation to acute leukemia
Myeloproliferative disorders
(CML, PV, ET, Myelofibrosis)
Classified based on dominant myeloid cell produced
Hyperuricemia and gout due to high turnover of cells
Characteristic Basophilia
Chronic Myeloid Leukemia (CML)
Increased mature myeloid cells
t(9:22)
Chronic Myeloid Leukemia (CML)
Philadelphia chromosome -> BCR-ABL fusion -> ^tyrosine kinase activity
LAP-
Imatinib treatment
Chronic Myeloid Leukemia (CML)
Blocks tyrosine kinase activity
Enlarging Spleen suggests progression to AML (2/3) or ALL (1/3)
LAP-
JAK2 kinase mutation
Polycythemia Vera (PV) Essential Thrombocythemia (ET) Myelofibrosis - 50%
Blurry Vision and HA
Risk of venous thrombosis
Flushed face (plethora)
Itching post bath
Polycythemia Vera (PV)
Venous thrombosis in hepatic vein, portal vein, dural sinus
Itching due to histamine release from mast cells
EPO decreased
Hydroxyurea treatment
Polycythemia Vera (PV)
1st line tx Phlebotomy
2nd line tx Hydroxyurea
EPO decreased in PV
Increased risk of bleeding and/or thrombosis
JAK2 mutation
Essential thrombocythemia (ET)
Rarely progress to BM fibrosis or acute leukemia
No significant risk of hyperuricemia or gout
Excess PDGF
Myelofibrosis
causes BM Fibrosis
JAK2 mutation