Hematology Flashcards
acquired deficiency of metalloproteinase
Thrombotic thrombocytopenic purpura (TTP)
Treatment of choice for TTP
Plasmapharesis
Autoimmune platelet destruction; most common cause of thrombocytopenic purpura in childhood
Idiopathic thrombocytopenic purpura (ITP)
Bruising and petechial rash 1-4 weeks after infection
Idiopathic thrombocytopenic purpura (ITP)
Treatment for ITP
None (70% spontaneously resolve)
For severe thrombocytopenia: IVIg, platelet transfusion and prednisone
X-linked recessive deficiency of factor VIII
Hemophilia A
X-linked recessive deficiency of factor IX
Hemophilia B or Christmas Disease
Normal bleeding time
5-8 mins
Most common inherited bleeding disorder
Von Willebrand disease
Treatment for Von Willebrand Disease
Desmopressin (mild);
Factor VIII concentrate (severe)
Increased PTT
Normal PT, BT, plt ct
Hemophilia A and B
Increased PT, PTT
Normal PT, plt ct
Von Willebrand’s Disease
Increased PT, PTT, BT
Decreased plt ct
Disseminated Intravascular Coagulopathy (DIC)
Increased PT, PTT
Normal BT, plt ct
Vitamin K deficiency
Normal PT, PTT
Increased BT
Decreased plt ct
Idiopathic thrombocytopenic purpura (ITP)
Fever, anemia, thrombocytopenia, renal dysfunction, nervous system changes
Thrombotic thrombocytopenic purpura (TTP)
Low Hgb, 0 retic
Normal WBC and platelets
BM: absence of red cell precursors
Transient erythroblastopenia of childhood
Normochromic, normocytic Leucocytosis Dec Serum Fe Normal TIBC Low to normal transferrin Inc serrm Ferritin Fe sequestration to macrophages Blunted response to EPO
Anemia of Chronic Disease
Two types of aplastic anemia
Fanconi (congenital) anemia
Acquired aplastic anemia
rare autosomal recessive inherited BM failure assoc with cafe au lait, short stature, microcephaly, skeletal, renal & cardiac anomalies
Fanconi anemia
Macrocytosis ff by thrombocytopenia
then progressive pancytopenia
terminating in myelodysplastic syndrome
Fanconi anemia
Pancytopenia d/t destruction of stem cells by IFN gamma and TNF (also potent inhibitors of progenitor cells)
Acquired immune-mediated aplastic anemia
Marked depression or absence of hematopoietic cells with replacement of fatty tissue-containing reticulin cells
Acquired aplastic anemia
Granulocyte <500
Platelet <20,000
Retic ct <40,000
Severe aplastic anemia
Storage diseases that cause BM infiltration
Gaucher’s
Newmann-Pick
Cystine storage disease
Rare pure red cell aplasia in w/c erythroid progenitors and precursors are highly sensitive to death by apoptosis
Diamond-Blackfan syndrome
Macrocytic anemia
Low retic
Normocellular BM with selective paucity of eryhteoid precursors
Inc Hgb F, inc eADA
(+) malignant potential
Normal karyotype, 50% had physical anomalies
median age: 2 mos
Diamond-blackfan syndrome
2 alpha 2 gamma
Hgb F
2 alpha 2 beta/delta
Adult Hgb
4 betas
Hgb H
4 gamma’s
Bart’s Hgb
