Heme Flashcards

1
Q

When should you use a blood transfusion for anemia?

A

HgB < 7 or CAD (inc oxygen needs)

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2
Q

Conversion of HgB to HCT

A

HgB x 3 = HCT

**So when give 1 unit of packed red cells, HgB inc by 1 unit and HCT by 3 units

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3
Q

General Steps in Anemia Work Up

A

1- CBC

2- if low HgB/HCT look at reticulocyte count; >2% means compensating for hemolysis or loss

3- if reticulocytes elevated suspect blood loss (look for source) OR hemolysis (haptoglobin, LDH, bilirubin)

4- if reticulocytes not elevated then get MCV

* Microcytic - iron studies 
* Macrocytic - check Vit B12 and folate; if normal then suspect liver disease 
* Normocytic - consider other causes
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4
Q

Why should packed RBCs not be given w/ LR?

A

Ca causes coagulation

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5
Q

Causes, Labs and Tx of Iron Def Anemia

A

Causes - breastfed infants (human milk is low in iron), adolescents and pregnant women have inc demand

Labs - low ferritin (stores), low serum iron, high TIBC but low TIBC saturation

Tx - can do oral iron trial in menstruating women; if male or post-menopausal then must r/o bleed first

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6
Q

Side Effects of Oral Iron

A

Constipation (give colace)

Nausea

Dyspepsia

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7
Q

Gel Electrophoresis Findings in Thalassemias

A

Beta - inc HbF and inc Hg A2

Alpha - inc Hg H (if 3 deletions)

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8
Q

Sideroblastic Anemia

A
  • Microcytic
  • lead poisoning, pyridoxine deficiency, alcohol use, anything that causes defective production of protoporphyrin
  • See iron accumulation in mitochondria of BM
  • Treat underlying offender
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9
Q

Vit B12 Deficiency (findings, labs, poss causes, tx)

A
  • Also get glossitis and neuro sx
    • Posterior columns - loss of position and vibration sense
    • Lateral corticospinal tracts - upper motor neuron signs
    • Spinocerebellar - ataxia
  • B12 is absorbed in ileum
    • Affected by gastrectomy, Chron’s, pernicious anemia (autoimmune; dec intrinsic factor), fish tapeworm, poor diet, alcoholism
  • Inc methylmalonic acid and homocysteine
  • Hypersegmented neutrophils
  • Tx - monthly IM injection
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10
Q

Schilling Test

A

Schilling test for pernicious anemia - absorption improves w/ addition of intrinsic factor (otherwise general malabsorption)

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11
Q

Folate Deficiency (findings, poss causes, labs, tx)

A
  • May be related to methotrexate or phenytoin use, HD, inc demand (pregnancy), poor diet / alcoholism
  • No neuro deficits; normal methylmalonic acid
  • Tx - daily oral supplement

ASK CAGE QUESTIONS

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12
Q

Labs in Anemia of Chronic Disease

A
  • May be microcytic or normocytic

* high serum ferritin (high stores because iron stuck in macrophages); low serum iron, low TIBC

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13
Q

Causes of Aplastic Anemia

A
  • Meds - chloramphenicol, sulfonamides, gold, carbamazepine
  • Radiation
  • Viral infection - HPV, HepB, hep C, EBV, CMV, zoster, HIV
  • Chemicals - insecticides
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14
Q

Findings in Aplastic Anemia

A
  • Pancytopenia (dec RBCs - anemia, dec platelets - bleeding, bruising and dec WBCs - infections)
  • BM biopsy will show hypocellularity
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15
Q

Symptoms and Labs of Hemolytic Anemias

A
  • Additional Presentation - dark urine (hemoglobinuria if intravascular), hepatomegaly, splenomegaly, lymphadenopathy if chronic
  • Labs
  • elevated reticulocyte count b/c comp by inc RBC production
  • low haptoglobin (binds to hemoglobin so low haptoglobin means hgb destroyed)
  • high LDH (released)
  • high indirect bilirubin (heme by-product)
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16
Q

Blood Smear of Intravascular v Extravascular Hemolytic Anemia

A

Intravascular (in vessels) - schistocytes
-DIC, TTP, prosthetic valve

Extravascular (in spleen or reticulo system) -spherocytes
-G6PD, autoimmune, paroxysmal nocturnal, hereditary spherocytosis

17
Q

Hereditary Spherocytosis

A
  • spectrin defect so destroyed by spleen (loss of RBC membrane surface area but no dec in volume so take on sphere shape)
  • Inc osmotic fragility; RBCs burst in hypotonic saline at much higher oncotic pressure than normal
  • Direct Coombs negative
  • May need splenectomy
18
Q

G6PD Deficiency

A

Triggers - sulfonamides, nitro, primaqiune, dimercaprol, Favo beans, infections

Un-neutralized H2O2 denatures Hb –>Heinz body precipitates –> dec flexibility –> destroyed by spleen (bites missing)

Deficiency NADPH formation; do not test for G6PD levels in acute attack (will be false negative because cells w/o G6PD will be destroyed leaving only normal cells to test

19
Q

Autoimmune Hemolytic Anemia

A

Spherocytes

Warm - IgG

Cold - IgM

20
Q

Warm AIHA

A
  • IgG binds RBC membrane in warm temp
  • extravascular hemolysis (splenomegaly)
  • Causes - Lymphoma, CLL, malignancy, collagen vascular disease like SLE, alpha-methyldopa
  • Pos direct Coombs test - RBCs coated w/ IgG
  • Tx - steroids, splenectomy ot transfusions if needed, may replace folic acid
21
Q

Cold AIHA

A
  • IgM binds to RBCs at cold temp
  • intravascular hemolysis esp in liver
  • Causes - Mono or mycoplasma or idopathic (esp in elderly)
  • Coated w/ complement on Direct Coomb’s
  • Pos cold agglutinin titer
  • Tx - NO STEROIDS; avoid cold exposure, treat underlying cause
22
Q

Paroxysmal Nocturnal Hemoglobinuria (manifestations and special tests and tx)

A
  • deficiency in anchor for complement inactivating proteins on RBCs –> more susceptible to complement damage –> hemolysis at night (acidotic)
  • Also affects WBCs and platelets (pancytopenia)
  • Venous system thrombosis (Budd-Chiari - hepatic veins)
  • Flow cytometry shows no or low CD55, CD59
  • Ham Test - lysis when incubated w/ acidic serum
  • Sugar Water Test - mix pt serum w/ sucrose; hemolysis occurs
  • Tx - steroids +/- BM transplant
23
Q

Complications of Sickle Cell Disease (15)

A
  • Pigmented gallstones
    • Aplastic crisis w/ parvovirus B19
    • Pain crisis (self-resolved in 1 wk; give morphine)
    • Hand-foot syndrome - swelling in infants/kids from avascular necrosis of metacarpal and metatarsals
    • Acute chest - chest pain, infiltrates, resp distress, hypoxia
    • Autosplenectomy - infarcts; calcified
    • Avascular necrosis of joints (hip and shoulder)
    • Priapism - erection 30 min - 3 hr from vaso-occlusion
    • CVAs - cerebral thrombosis in kids
    • Optho infarcts, retinopathy, retinal detachment
    • Renal papillary necrosis - painless hematuria
    • Chronic lateral malleolus leg ulcers - dec flow to superficial veins
    • Abdominal crisis
    • Infection - encapsulated (SHiN); Salmonella osteomyelitis
    • Delayed growth and sexual maturation