Heme Flashcards
Most sensitive assay for IDA
Transferrin/ ferritin receptor assay
> 2: IDA
Best screening for hemochromatosis
Transferrin sat
Normal lab values: MCV TIBC Ferritin Iron Transferrin sat
Mcv: 80-100 Tibc: 220-240 Ferritin: > 20 Iron: 50-160 Transferrin sat: 20-40%
Dxtic for alpha thalassemia
DNA PCR analysis
Dxtic for beta thalassemia
Hgb electrophoresis
+ hgb A2
Clues on CBC for beta thalassemia
Looks like iron def (anemia, low mcv) but normal RDW and high RBC count
Sickle cell trait
Splenic sequestration
Hematuria
Sickle cell anemia
Vaso-occlusive dse
Infxn w/ encapsulated
Sickle cell dse
Vaso-occlusive
Avacular necrosis
Retinal infarcts
Leading cause of death in sickle
Acute chest crisis
Shown to produce cure in sickle
Peripheral blood stem cell transplant
Sickle cell w/ stroke in evolution
Exchange transfusion
Most common cause of osteomyelitis in sickle
Salmonella
Characteristics of megaloblastic anemia
Inc LDH and bilirubin
Dec retic count
Oval macrocytes, hypercellular marrow w/ megaloblasts
T/F Folic acid will inc platelets
T (falsely elevated)
T/F all chronic hemolytic syndromes should receive folic acid
T (falsely normal)
Causes of folic def
A folic drop
Alcohol/ folate antagonists/ OCP/ low intake/ lnfxn/ celiac sprue/ dilantin/ rel folic def/ old age/ pregnancy
How to differentiate b12 fr folic def
Folic: high homocysteine and normal methylmalonic acid levels
B12: high himocysteine and high methymalonic acid levels (MM acid levels rise before b12 goes down)
T/F vit b6 def will also have high homocysteine levels
T
Normal b12 level
200-800
Megaloblastic anemia w: hyposegmented PMNs
Myelodysplastic syndrome
MDS tx
55 - azacitidine
epo 500 - antithymocyte Ig + cyclosporine
Unable to tolerate ATG- lenalidomide
5q deletion- lenalidomide
Dxtics for hemochromatosis
Screening: Transferrin sat > 45, ferritin > 1000
Dxtic: HFE gene testing
To find out extent of iron overload:
Liver biopsy
Tx for hemochromatosis
Phlebotomy
Deferoxamine
Aplastic anemia tx
50 ATG + cyclosporine
Refractory to ATG- alemtuzumab
Burr and spur cells
Burr- renal
Spur- liver
Warm antibody
Ag: Rh
Ab: IgG +/- c3
Etio: sle, cll, lymphoma, drugs
Tx: steroids, danazol, splenectomy, rituximab
Cold antibody
Ag: iAg
Ab: IgM, C3
Etio: quinidine, lymphoba, viral
Tx: no role for steroids
Cyclophosphamide
Chlorambucil - rituximab
PNH dxtic
CD 55, 59
Tx for PNH
BM transplant
Eculizumab dec transfusion need
Dxtic for hereditary spherocytosis
Osmotic fragility test
Tx for hereditary spherocytosis
FA, splenectomy
Defect in spherocytosis
Spectrin membrane defect
Bite/ blister cells
G6PD
When to repeat G6PD levels after attack
2-3 months
TTP
Fever, renal, pancytopenia, autoimmune hemolytic anemia, neuro (headache, forgetfulness)
T/F Pt, ptt is abnormal in TTP
F
Tx for TTP
Plasmapheresis FFP (bec it has adamts 13)
Bloody diarrhea, renal failure, hemolytic anemia
HU
Tx: supportive
Fever, chills, sob w/in one hr of BT
Major hemolytic rxn - ABO incompatibility
Hematuria, jaundice a week after BT
Late transfusion rxn- Rh incompatibility
Hx of Urticaria and allergies needs BT
Washed PRBC
Fever and chills every time transfused
Leukocyte reduced PRBC
Fever, chills, sob, angioedema minutes into BT
IgA deficiency
Tx: washed PRBC