Hemonc Flashcards
african american pt with low hematocrit and high reticulocyte count, recurrent episodes of abd pain that resolve with hydration. Ddx
sickle cell anemia
acte chest syndrome
vaso-occlusive crisis localized to the pulmonary vasculature that occurs in sickle cell pt
autosplenectomy
sickle cell pt. due to vaso-occlusive crises that cause repeated infarctions. Spleen is shrunken,brown discoloration and fibrotic.
the primary mechanism of iron deficiency is
blood loss, especially in GI tract
cause of hypochromic, microcytic anemia
iron deficiency
total body iron content is regulated by
Hepcidin from hepatic parenchymal cells
X linked coagulopathy, intramuscular hemorrhage, hemorrhage into large joints, prolonged bleeding following surgery.
Hemophilia A
signs of disorder of secondary hemostasis
bleeding from joints and muscles, bleeding after surgery
coagulopathy with x-linker inheritance and PTT pronlongation
Hemophilia A: only males can get it
deficiency of CD55 and CD59, hemolytic anemia, hypercoagulable state and decreased blood counts
Paroxysmal nocturnal hemoglobinuria
bone marrow aspiration in aplastic anemia
dry tap
easy bleeding form mucosal surfaces including gingivae, nasal mucosa, GI tract and menorrhagia.
Von willebrand disease
inheritance of von willebrand disease
Autosomal dominant with variable incomplete penetrance
pt with severe B thalasemia receives regular blood transfusions. Lymph node biopsy revelas phagocytic cells with golden yellow cytoplasmic granules that are composed of
Hemosiderin
G6PD inheritance
X linked recessive, males are affected and females r carriers
Hereditary spherocytosis inheritance
Autosomal dominant
pt with increased plasma volume, low EPO levels, pruritis after hot showers
Polycythemia vera
mutation in polycythemia vera
JAK2 making bone marrow more sensitive to growth factors
enzyme affected in acute intermittent porphyria
porphobilinogen deaminase
enzyme affected in porphyria cutanea tarde
uroporphyrinogen decarboxylase
enzymes affected in lead poisoning
ferrochalatase and ALA dehydrogenase (RLS)
cryoprecipitate
fibrinogen, factor VIII, factor XIII, vWF and fibronectin
Fresh froxen plasma
coagulation factors
treatment of DIC, cirrhosis and warfarin overdose
fresh frozen plasma
translocation 9:22
CML, bcr-abl