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Sx of anemia depends on 3 factors, each play a role:
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1. Hgb level: the less, the worse (<8 = pallor / <4= ischemia)
2. Cardiac reserve, a.k.a comorbidities: Athelete compensates while pt w/ HF + COPD dont
3. Change over time: if Hgb is changing fast over time → worse sx
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Microcytic Anemia
· In case u did iron studies and they are ⓝ, what’s next? go for electrophoresis
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Iron deficiency anemia
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Alpha-thalassemia minor
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Beta-thalassemia minor
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Anemia of chronic disease
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MCV
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↓
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↓
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↓
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ⓝ / ↓
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RDW
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↑
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ⓝ
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ⓝ
|
|
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RBCs
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↓
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ⓝ
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ⓝ
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Peripheral smear
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Microcytosis, hypochromia
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Target cells
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Target cells
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Normocytic / Microcytic
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Iron
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↓
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ⓝ
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ⓝ
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↓
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TIBC
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↑
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ⓝ
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ⓝ
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↓
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Ferritin
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↓
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ⓝ
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ⓝ
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↑
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Transferrin
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↓
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ⓝ
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ⓝ
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ⓝ / ↓
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Response to iron supplementation
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↑ Hemoglobin
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ø
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ø
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ø
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Hemoglobin electrophoresis
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ⓝ
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ⓝ
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↑ Hemoglobin A2
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ø
|
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Disorder (genotype)
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Hgb Electrophoresis
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Anemia Severity
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a-thalassemia
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SILENT
(1 Gene deleted)
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ⓝ
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Asx
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|
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Trait
(2 Genes deleted)
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ⓝ
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Mild
|
|
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Hgb H disease
(3 Genes deleted)
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· 5-30% Hgb H (in adults)
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Chronic hemolysis
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|
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Fetal hydrops
(4 Genes deleted)
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· ❌ Absent Hgb A, Hgb F & Hgb A2
· Hgb barts & Hgb H are present
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Death in utero
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b-thalassemia
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Trait
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⤴️ Hgb A2
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Mild
|
|
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Intermediate
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⤴️ Hgb F
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Moderate
|
|
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Major
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· ❌ Absent Hgb A
· ✓ Hgb F & Hgb A2 present
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Severe
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Lead poisoning in adults
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RF
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Occupational exposure (eg, lead paint,
batteries, ammunition, construction)
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Clx
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· GI (abdominal pain, constipation, anorexia)
· Neurologic (cognitive deficits, peripheral neuropathy)
· Hematologic (anemia)
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Labs
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· Anemia
· HyperURICEMIA
· ⤴️ venous lead level
· ⤴️ serum zinc protoporphyrin level
· Basophilic stippling on peripheral smear
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Sidroblastic Anemia
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Path
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· Defective Heme synthesis
· Lead
· INH SE (B6 def)
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S/S
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Microcytic Anemia + GI + Neuro
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Dx
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· CBC: Microcytic anemia
· Iron studies: ⤴️ Iron / ⤵️ TIBC
· BM: Sideroblasts
· Smear: Basophilic stippling
· ⤴️ Lead venous level
· ⤴️ zinc protoporphyrin
· ⤴️ Uric Acid
|
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Tx
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If related to INH: administer B6
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Macrocytic Anemia
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Common causes of Macrocytic Anemia
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⤴️ MCV >100 fL
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|
· Folate deficiency
· Vitamin B12 deficiency
· Myelodysplastic syndromes
· Acute myeloid leukemias
· Drug-induced (eg, hydroxyurea, zidovudine, chemotherapy agents)
· Liver disease
· Alcohol abuse
· Hypothyroidism
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|
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Folate
|
B12
|
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Clx
|
Anemia
|
· Anemia
· Neuropathy
· Dementia
|
|
Pathology
|
· Chronic Hemolysis (SCA)
· Poor dietary intake (alcoholic)
· Malabsorption (Gastric bypass)
· Medications (MTX)
|
· Pernicious anemia
· Vegans
|
|
Labs
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· Poor reticulocyte response (low to normal)
· Hypersegmented Neutrophils
· Low serum folate
|
· Hypersegmented Neutrophils
· Low serum B12
|
|
Homocystine
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⤴️
|
⤴️
|
|
MMA
|
ⓝ
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⤴️
|
|
Tx
|
Folic acid supplementation
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B12 supplementation
|
|
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Fanconi Anemia
|
Diamond-Blackfan
|
|
Path
|
· AR
· DNA Repair defect —+ fucked up BM (Pancytopenia)
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Congenital erythroid aplasia
|
|
Clx
|
· Craniofacial abnormalities
· abnormal thumbs
· radial bone hypoplasia
· Deafness
· Macrocytic anemia
|
· Craniofacial abnormalities
· Triphalangeal thumbs (thumb has 3 phalanges instead of 2)
· ⤴️ Risk of malignancy
|
|
Dx
|
PANCYTOPENIA
Chormosomal breaks on genetic analysis
|
· Macrocytic anemia
· ⤵️ Reticulocyte
· ⓝ PLT / WBC
|
|
Tx
|
BM Transplant
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· Corticosteroids
· RBC transfusions
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Normocytic Anemia
|
G6PD deficiency
|
|
#
|
· Hemolytic anemia due to oxidative stress (infection, sulfa drugs, fava beans)
· X-Iinked: Asian, African, or Middle Eastern descent
|
|
S/S
|
· Pallor & fatigue
· Dark urine, jaundice & icterus
· Abdominal/back pain
|
|
Dx
|
· ⤵️ hemoglobin, ⤵️ haptoglobin,
· ⤴️ bilirubin & LDH
· Peripheral smear: bite cells & Heinz bodies
· ⊖ Coombs test
· ⤵️ G6PD activity level (may be ⓝ during attack)
|
|
Tx
|
Remove or treat responsible agent/condition
Provide supportive care
|
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Drugs that trigger hemolysis in G6PD
|
|
Avoid
|
· Dapsone
· Isobutyl Nitrite
· Nitrofurantoin
· Primaquine
· Rasburicase
|
|
Use w/ caution
|
|
· Acetaminophen
· Aspirin
· Chloramphenicol
· Chloroquine
· Colchicine
|
· Glyburide
· Isoniazid
· L-dopa
· Quinine
· Sulfamethoxazole
· Trimethoprim
· Vitamin K
|
|
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Hereditary Spherocytosis
|
|
Features
|
· AD
· Northern European descent
|
|
S/S
|
Triad ∆:
· Hemolytic anemia
· Jaundice
· Splenomegaly
|
|
Dx
|
· ⤴️ MCHC
· ⊕ Spherocytes
· ⊖Coombs (vs Autoimmune hemolytic anemia)
· ⤴️ Osmotic fragility on acidified glycerol lysis test
· Abnormal eosin-5-maleimide binding test
|
|
Tx
|
· Folic acid supplementation
· Blood transfusion
· Splenectomy
|
|
Autoimmune Hemolytic Anemia
|
|
|
Warm
|
Cold
|
|
Etiology
|
· Drugs (Penicillins)
· Viral infections
· Autoimmune (SLE)
· Immunodefeciency states
· Lymphoproliferative (CLL)
|
· Infections (Mycoplasma, Infectious mononucleosis)
· Lymphoproliferative
|
|
S/S
|
· Asx → Life-threatening
· ⊕Direct coombs
· ⊕IgG
|
· Anemia Sx
· Livedo reticularis & Acral cyanosis w/ exposure to cold
· ⊕Direct coombs
· ⊕ IgM
|
|
Tx
|
· Corticosteroids
· Splenectomy if REFRACTORY
|
· Avoid cold temp
· Retuximab
|
|
Complications
|
· Thromboembolism
· Lymphoproliferative diseases
|
· Ischemia & gangrene
· Lymphoproliferative diseases
|
|
Aplastic anemia
|
|
Features
|
Path BM failure due to hematopoietic SC deficiency (CD34+)
|
|
S/S
|
• Autoimmune
• Parvovirus 819, EBV
• Drugs (eg, carbamazepine, chloramphenicol, sulfonamides)
• Exposure to radiation or toxins (eg, benzene, solvents)
|
|
Lab
|
Pancytopenia
· Anemia (fatigue, weakness, pallor)
· Thrombocytopenia (mucosal bleeding, easy bruising, petechiae)
· Leukopenia (recurrent infections)
Biopsy: Hypocellular bone marrow with fat and stromal cells
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Fast HY Review 