Hematology 2 Flashcards
Mutation in hereditary spherocytosis
spectrin, ankyrin, Band 3, protein 4.2
Band 3 mutation
hereditary spherocytosis
SE asian ovalocytosis
CBC abnormality in hereditary spherocytosis
increased MCHC
DDX of spherocytes
HS, immune mediated hemolysis
Mutation in hereditary elliptocytosis
Spectrin
Spectrin mutation
hereditary elliptocytosis
DDX if elliptocytes <25%
iron deficiency
megaloblastic anemia
MDS
Myelophthesis
Hereditary pyropoikilocytosis
variant of HE
hemolysis at 45C
transient in neonates
SE Asian ovalocytosis
Band 3 mutation
SE Asian ovalocytosis protects against
P. vivax
smear findings in Rh null phenotype
stomatocytes
Dz with thrombosis after splenectomy
hereditary stomatocytosis
name of precipitated Hgb seen in RBCs
Heinz bodies
Peripheral smear in G6PD def
Heinz bodies, poikilocytosis, spherocytes, bite/blister cells
Smear in pyruvate kinase def
ecchinocytes (burr cells) mostly after splenectomy
Autohemolysis test
Hereditary Spherocytosis (corrects with glucose) Pyruvate kinase def (does not correct)
Fluorescent spot test used for
pyruvate kinase def
Congenital dyserythropoietic anemia II
HEMPA, + acidified serum test (heterologous only)
Disorder with HAM + only with heterologous serum
Congenital Dyserythropoietic Anemia II (HEPAS)
Disorder with HAM+ with both heterologous and autologous serum
Paroxysmal Nocturnal Hemolobinuria
Increased density of i-antigen seen in
CDA II
Blackfan-Diamond
Location of PIG-A gene
X-chromosome
Decreased in PNH
CD55, CD59, AchE, CD16, CD48
Sucrose hemolysis test + in
PNH
LAP score in PNH
decreased
Labs in sideroblastic anemia (haptoglobin, transferrin saturation, ferritin, Iron, LDH)
increased iron, transferrin saturation, ferritin, LDH
decreased haptoglobin
Causes of acquired sideroblastic anemia
MDS, meds, radiation, EtOH, Pearson syndrome, Copper deficiency
Pearson syndrome
sideroblastic anemia with pancreatic insuffiency
congenital pure red cell aplasia
Blackfan-Diamond syndrome
treatment of Blackfan-Diamond
corticosteroids
Type of thymoma assd with red cell aplasia
spindle cell/meduallary/typeA