Goljan Rbc Disorders

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Stimuli for EPO

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hypoxemia
left-shifted OBC
high altitude

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EPO is made

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in the endothelial cells of the peritubular capillaries

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corrected retic count

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effective if > or = 3%
ineffective is < 3%
(actual Hct/45 * retic count)

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fetal-Hb and OBC

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left shit

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lab values in thalassemia

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Hb, Hct are decreased
RBC count increased

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O2 sat and arterial PO2 in anemia

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both are normal

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increased MCHC

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seen in spherocytes

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RDW

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reflects variation in size of RBCs in the peripheral blood
Fe def. anemia is the only microcytic anemia with increased RDW

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serum ferritin

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decreased in fe def
increased in ACD, iron overload diseases

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serum iron levels

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decreased in Fe def, ACD
increased in iron overload diseases

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TIBC and transferrin

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increased TIBC = increased transferrin and vice versa

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decreased ferritin stores

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cause increased synthesis of transferrin/TIBC
Fe def = increased transferrin

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increased ferritin stores

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decreased transferrin/TIBC
ACD, iron overload diseases

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decreased %sat

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Fe def
ACD

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increased %sat

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iron overload diseases

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HbA

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2alpha/2beta

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HbA2

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2alpha/2delta

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HbF

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2alpha/2gamma

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iron def. most often caused by

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bleeding

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most commona anemia in hospitalized patients

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ACD

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hepcidin

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increased mac iron stores
enters macs and prevents release of iron to transferrin

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Hb electrophoresis in alpha-thal trait

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is normal

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Beta-Thal minor

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DNA splicing defect
decreased HbA
increased RBC count, HbA2, HbF

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Beta-thal major

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nonsense mutation --> stop codon
no HbA, increased HbA2, HbF

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sideroblastic anemia

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defect in heme synthesis in the mitochondria
ringed siderblasts
due to booze, B6 def, and Pb poisoning

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most common cause of B6 def

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INH

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Pb poisoning

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paint, batteries
coarse basophilling stippling
Pb deposits in epiphyses leading to growth problems

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phenytoin and folate

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folate is coverted to monoglutamates which are reabsorbed in the jejunum by intestinal conjugase
phenytoin inhibits intestinal conjugase leading to decreased folate absorption

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alcohol and oral contraceptives

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block reabsorption of folate in the jejunum

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increased homocysteine

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folate and vit B12 def

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most sensitive test for B12 def

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increased methylmalonic acid

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signs of volume depletion

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decreased blood pressure
increased pulse

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anemia of CRF

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decreased EPO
normocytic
burr cells
throbocytopenia
defect in platelet aggregation that is reversible with dialysis

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increased RBC osmotic fragility

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hereditary spherocytosis

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PNH

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loss of anchor for DAF which normally neutralizes complement attached to RBCs, platelets, and PMNs

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PNH lab tests

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screen: sucrose hemolysis test --> sucrose enhances complement destruction of RBCs
confirm: acidified serum test --> activates the alternative pw causing hemolysis

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target cells

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excess RBC membrane
sign of hemoglobinopathy or alcohol excess

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dactylitis

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painful swelling of the hands and feet in infants with SCA (around 6-9 months) due to bone infarcts

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acute chest syndrome

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vaso-occlusion of pulmonary capillaries
chest pain, lung infiltrates, and hypoxemia
most common cause of death in adults with SCA

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PK def

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normocytic anemia
RBCs with thorny projections
increased 2,3-BPG right shifts OBC

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direct antihuman globulin test (DAT)

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most important marker for immune hemolytic anemia

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malaria and hemolysis

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intravascular hemolysis correlates with fever spikes