hemaped Flashcards
causes of microcytic anemia
(TAILS) Thalassemia; Anemia of chronic dse; Iron deficiency; Lead poisoning; sideroblastic anemia
causes of normocytic anemia
anemia of chronic dse; uremia; hypothyroidism; bone marrow failure(aplastic anemia)
causes of macrocytic anemia
folate deficiency; vit B12 deficiency; drug and alcohol-induced anemia
anemia caused by failure or autoimmune destruction of myeloid stem cells
aplastic anemia
viruses that cause aplastic anemia
parvovirus B19; hepatitis viruses; HIV; Epstein-Barr virus
causes of aplastic anemia
idiopathic; viruses; alklylating and antimetabolite agents; chloramphenicol; insecticides; benzene; radiation
typical peripheral blodd smear findings in aplastic anemia
hypocellularity and pancytopenia
aplastic anemia with microcephaly; microphthalmia; hearing loss; limb anomalies(absent radii and thumbs)
Fanconi’s anemia
aka Diamond-Blackfan syndrome or congenital hypoplastic anemia
pure red cell aplasia
absence of erythrocyte precursor cells on bone marrow aspirate
pure red cell aplasia
most important sign in iron deficiency anemia
pallor
clinical manifestations in iron deficiency anemia
asymptomatic; lethargic; irritable; pale; easily fatigued; high output cardiac failure; koilonychia(spoon nails); pica
low retic count; microcytic; hypochromic RBC’s; high RDW; low serum iron and ferritin; high TIBC
Iron deficiency anemia
due to decrease in alpha-globulin chain synthesis; dec retic count; microcytic; hypochromic; normal RDW; taget cells; Heinz bodies on PBS
alpha thalassemia
no beta-globin production; anemia at 6mos; splenomegaly;extramedullary hematopoiesis
Beta-thalassemia major(Cooley’s anemia)
asymptomatic carriers of beta-thalassemia gene; mild or no anemia
Beta-thalassemia minor
definitive diagnosis of thalassemias
Hb electrophoresis
etiologies of hemolytic anemias
intrinsic RBC defects; autoimmune destruction; intravascular destruction
defect in membrane spectrin or ankyrin
spherocytosis
confirmatory test for spherocytosis
osmotic fragility test
curative treatment to spherocytosis
splenectomy
lack of enzyme needed by RBC to defend against oxidative stress leading to RBC destruction
G6PD deficiency
typical PBS finding in G6PD deficiency
Heinz cells; blister cells; bite cells; ghost cells (implying intravascular hemolytic anemia)
triggers of G6PD deficiency
FPAINNS Fava beans; Primaquine; ASA; Isoniazid; NSAIDS; Nitrofurantoin; Sulfa drugs
episodes of painful crisis due to hypoxic tissue injury from microvascular occlusions
sickle cell dse
radiologic findings in sickle cell dse
new bone formation on the external aspect of the skull (Crew cut or Hair on end appearanceon the skull radiographs)
definitive diagnosis in sickle cell dse
HbS on Hb electrophoresis
autoimmune platelet destruction; most common cause of thrombocytopenic purpura in children
ITP
five cardinal symptoms of TTP
FAT RN Fever; Anemia; Thrombocytopenia; Renal dysfunction; Nervous system damages
due to an acquired deficiency of a metaloproteinase (ADAMTS 13) that is responsible for cleaving the high molecular weight multimers of vWf
TTP
X-linked recessive deficiencies of Factor VIII or Factor IX
Hemophilia
Dx of hemophilia
normal BT PC and PT; prolonged PTT; decreased factor levels
most common inherited bleeding disorder
von Willebrand disease
treatment of mild bleeding in type I vWd
Desmopressin(causes release of vWf from endothelial stores)
Tx for severe vWd
Factor VIII concentrates which contain high vWf Ag
