Hematology Flashcards
Disorders of primary hemostasis
Platelet: quantitative (thrombocytopenia) and qualitative (functional defects)
Von Willebrand Disease
Disorders of secondary hemostasis
Congenital: hemophilia A and B, rare factor deficiencies
Acquired: Vit. K deficiency, liver disease
von Willebrand disease
Most common inherited bleeding disorder
Def: mutations in the gene coding for von willebrand factor results in dysfunction of platelet adhesion or the dysfunction of carrying of factor VIII and not protecting it
S/S: epistaxis, dental bleeding, menorrhagia, bruising, prolonged bleeding (primary hemostasis), severly affected pts may have joint and soft tissue hemorrhages
Dgx: vW panel: low vW antigen, low factor VIII, low ristocetin cofactor activity (protein doesn’t func);
other tests: abnormal platelet func testing, NORMAL INR, aPTT, and platelet count
Txt: avoid drugs that inhibit plt func, DDAVP, antifibrinolytics, plasma derived VIII/vWF, cryoprecipitate
Hemophilia
Def: congenital bleeding disorder caused by deficiency of coag factor VIII (type A, classic) or IX (type B, Christmas) (both located on X chromosome)
S/s: clinical phenotypes are indistinguishable; severity of bleeding depends on the factor level; joint, soft tissue, deep muscle, and intracranial bleeding
Dgx: Markedly prolonged PTT, NORMAL INR and plt count and func. measure specific factor levels
Tx: avoid drugs that impair plt func, factor VIII or IX concentrates, prophylactic factor replacement
Sickle-cell dx
Def: inherited disorder of erythrocytes
SS: pain Sickle-shaped rigid blocks vascular flow 10-20 day lifespan
Eval:
peripheral blood smear
Txt:
Hydroxyurea
Leukemia
split into acute or chronic and myeloid or lymphoid
S/s: fatigue, infection, mild bleeding symptoms; abnormal CBC of pancytopenia or marked leukocytosis
Dgx: detailed Hx and PE; labs: CBC, renal and liver function, coags, peripheral blood immunophenotyping; Bone marrow and biopsy; imaging studies
Acute: blocks mature B cells from being made; meyloid stem cell side is unaffected
Tx: chemo
HIV summary
Def: retrovirus that does reverse transcriptase in host cell and convert their RNA into DNA
Cause: fluid, blood, tissues from infected person
SS: Fever Fatigue Rash HA Viral load - HIV replication rate CD4 count - immunologic damage
Eval: One of these: Serum antibody Saliva and urine antibody test Rapid tests - SUDS finger stick - OraQuick
Confirm with this: Western blot assay
Iron deficiency anemia
SS: fatigue dyspnea dysphagia pallor
Eval: CBC Microcytosis low ferritin elevated TIBC
Txt;
200 mg elemental iron/day
B12 Deficiency anemia
SS: fatigue dyspnea palpitations pallor jaundice glossitis neurologic symptoms
Eval:
CBC
macrocytosis
low retic count
Txt:
1000 mcg qd x 1 wk
1000 mcg q week
1000 mcg q month
Folic Acid anemia
SS: fatigue dyspnea palpitations jaundice glossitis
Eval:
macrocytosis
Txt:
1-5 mg po qd x 4 mths
