Hematology Flashcards
Microcytic Anemias (MCV <80)
- Thalassemia
- Iron Deficiency
- Lead poisoning
- Sideroblastic anemia
- Anemia of chronic disease
Normocytic anemia (MCV 80-100)
- Anemia of chronic disease
- Hemolytic anemia
- Hemorrhage
Macrocytic anemia (MCV >100)
- Folate deficiency
- Vit B12 deficiency
- Liver disease
- Alcohol abuse.
Iron deficient anemia
Pt
Dx
Management
- Pt
- fatigue, SOB, tachycardia, pallor.
- Ice pica.
- restless leg syndrome
- angular cheilitis
- Dx
- low H&H, low MCV, low MCH, MCHC.
- Iron study: Low iron, High transferrin, low ferritin, low % sat.
- Tx
- iron supplementation
- if >50, perform colonoscopy to rule out GI bleed.
Lead Poisoning
- Pt
- microcytic anemia.
- abd pain, constipation
- joint, muscle pain.
- HA, peripheral neuropathy.
- Dx
- Low H&H, low MCV
- High serum Iron
- Basophilic stippling on peripheral smear
- Tx
- eliminate source of exposure
- Chelation
- Adults: EDTA, succimer
- Children: dimercaprol
Sideroblastic anemia
- Etiology
- genetic.
- EtOH abuse or isoniazide
- Pt
- microcytic anemia, fatigue, pallor, SOB
- Dx
- Bone marrow biopsy w/ ringed sideroblasts.
- basically the only blood findings will be microcytic anemia.
-
Tx
- Genetic: Vit B6
- Acquired: stop EtOH, isoniazide.
- RBC transfusions, EPO
- Deferoxamine, phlebotomy
Alpha Thalassemia
- Minima
- 1 globin allele
- Asymptomatic
- Minor (trait)
- 2 globin allele
- asymptomatic
- Hemoglobin H disease
- 3 allele.
- microcytic anemia, chronic hemolysis
-
Hydrops fetalis
- 4 allele.
- Hgb Bart (4 gamma globins)
- fetal edema and intrauterine death. Mimics a Lt shift.
Beta thalassemia
- Minor
- 1 allele
- low beta gloin.
- High Hgb A2 (alpha, delta)
- Minimal disease
- Major
- 2 allele
- (+) Hgb F (alpha, gamma + Hgb A2
Sickle Cell Disease
Genetics
Pt
- Genetics
- Glu to Val mutation at position 6 of chr 11.
- AR inheritance.
- Pt
- Acidosis, hypoxia, dehydration cause cell sickling which leads to hemolysis and vaso-occlusive crisis.
- acute chest syndrome
- stroke
- Dactylitis (finger swelling)
- bone infarction, osteonecrosis
- Priapism
- Splenic sequestration –> auto-splenectomy
- Parvovirus B19 leads to aplastic crisis (where all cell lines are low).
Sickle Cell disease
Dx
Tx
- Dx
- anemia
- sickled RBC peripheral smear
- Hgb electrophoresis -> Hgb S + Hgb F
- Skull xray = hair on end appearance
- Tx
- IVF, supplemental O2, opioid analgesics
- Folic acid to supplement RBC production
- Hydroxyurea
- Immunize: pneumococcal, H. influenza B, Meningococcal, influenza.
- < 5: prophylactic penicillin.
Diagnosis of Hemolytic anemia
- Low H&H
- Normal MCV
- High indirect bili
- High LDH
- Low serum haptoglobin.
- High retic count.
Causes of Extrinsic Hemolysis
-
Microangiopathic anemia
- RBC death by narrow lumen passage.
- (+) schistocytes
-
Macroangiopathic anemia
- Mechanical forces shear RBC.
-
RBC infection
- malaria, babesiosis
-
Autoimmune
- (+) direct coombs test.
Autoimmune hemolytic anemia
- Cold agglutinins
- Ab to RBC < 4degrees.
- IgM. Seen with EBV, mycoplasma infection.
- Warm agglutinins
- Ab to RBC at normal body temp.
- IgG. HIV, EBV, Lupus, Cancers.
- Drug-induced
- cephalosporin
- penicillin
- NSAID
- methyldopa.
Treatment of autoimmune hemolytic anemia
- Cold agglutinins
- avoid cold temps
- Warm agglutinins
- Steroids
- Rituximab (monoclonal antibody against B cells)
- Splenectomy
Hereditary spherocytosis
Genetic defect in RBC membranes and cytoskeleton
- Pt
- splenomegaly, jaundice
- high risk aplastic anemia w/ parvovirus B19 infection
- Dx
- anemia w/ reticulocytosis and high MCHC.
- (+) spherocytes.
- (+) osmotic fragility test
- Tx
- Folic acid 1mg QD
- RBC transfusion if severe anemia
- Splenectomy –> (+) Howell-Jolly bodies.
G6PD deficiency is exacerbated by what?
Spleen Purges Nasty Inclusions From Damaged Cells
- Sulfonamides
- Primaquine
- Nitrofurantoin
- Isoniazide
- Fava bean
- Dapsone
- Chloroquine.
Diagnostic tests that indicate G6PD deficiency
- (+) Heinz bodies: precipitation of oxidized Hemoglobin in RBC
- (+) Degmacytes: deformed RBC following removal of Heinz bodies by spleen.
Urticarial transfusion reaction
Timing
Cause
Findings
Tx
- Timing
- during transfusion
- Cause
- IgE antibodies against the donor plasma
- Findings
- Urticaria
- Tx
- diphenhydramine
- stop transfusion
Anaphylactic transfusion reaction
Timing
Cause
Findings
Tx
- Timing
- seconds to minutes after start
- Cause
- Anti IgA/IgG antibodies in patient with IgA deficiency
- Findings
- Shock, HoTN, angioedema, respiratory failure.
- Tx
- Epinephrine, IVF, O2
Non-Hemolytic Febrile transfusion reaction
Timing
Cause
Findings
Tx
- Timing
- 1-6hr s/p
- Cause
- Cytokines produced by donor cells
- Findings
- fever, chills, malaise
- Tx
- Acetaminophen
Acute hemolytic transfusion reaction
Timing
Cause
Findings
Tx
- Timing:
- during transfusion
- Cause
- ABO incompatibility
- Findings
- Hemolysis, fever, chills, tachycardia, tachypnea, HoTN.
- Tx
- aggressive supportive therapy
Delayed hemolytic transfusion reaction
Timing
Cause
Findings
Tx
- Timing
- 2-10 day s/p
- Cause
- Anti-Kidd or anti-D antibodies
- Findings
- Mild fever, hemolysis, high indirect bili
- Tx
- None
Aplastic Anemia
Etiology
Pt
Dx
Tx
- Etiology
- radiation exposure
- Rx: chemotherapy, chloramphenicol, sulfonamides, phenytoin
- parvovirus B19 infection
- Pt
- Anemia, Leukopenia, Thrombocytopenia
- Dx
- (+) anemia, leukopenia, thrombocytopenia (>50% decrease of normal baseline)
- Bone marrow Bx = hypocellular and fat infiltration
- Tx
- bone marrow transplant
- Immunosuppressants.
B12 deficiency
- Etiology
- pernicious anemia.
- Gi bypass/resection (terminal ileum)
- strict vegans w/ no meat/dairy intake.
- Pt
- anemia
- dementia, Dorsal column dysfunction
- atrophic glossitis (tongue inflammation w/ loss of papillae)
- Dx
- MCV > 100
- low B12.
- High MMA, High Homocysteine
- Tx
- IM vit B12 injection
- oral B12 supplements.
Folate deficiency
- Etiology
- malabsorption: alcoholism, no leafy greens
- Pt
- anemia.
- atrophic glossitis
- NO neurological deficits.
- Dx
- MCV> 100
- MMA normal
- Tx
- folate supplements
- evaluate for concurrent B12 deficiency