STEPTRACKERHEMATOLOGY | PATHOLOGY + BIOCHEMISTRY | HIGH-YIELD REVIEW

Macrocytic Anemias

Big red cells: megaloblastic (DNA synthesis fails — B12, folate, drugs) or nonmegaloblastic (alcohol, liver, thyroid, marrow disease).

FOCUS ON
MMA separates the two deficiencies: ↑ in B12 deficiency, normal in folate deficiency (homocysteine is ↑ in both). Only B12 deficiency causes neurologic damage (subacute combined degeneration), and folate can correct the anemia while letting nerve damage progress. Pernicious anemia is the classic cause.
TOPIC
DETAIL
MECHANISM
CLUE
Megaloblastic change
the picture
Large oval RBCs, hypersegmented neutrophils (≥ 5 lobes); can cause pancytopenia.
Thymidine synthesis ↓ → nucleus matures slower than cytoplasm; ineffective erythropoiesis
↑ LDH and indirect bilirubin from marrow cell death
Folate deficiency
Poor diet (alcohol, elderly), ↑ demand (pregnancy, hemolysis), drugs (methotrexate, trimethoprim, phenytoin). Stores last ~3–4 months.
Absorbed in jejunum
↑ homocysteine, normal MMA, no neuro signs; pregnancy → neural tube defects
B12 deficiency — causes
Pernicious anemia (most common), gastrectomy, strict vegan diet, ileal resection or Crohn, fish tapeworm, pancreatic insufficiency, metformin, long PPI use, nitrous oxide.
Needs acid + intrinsic factor; absorbed in terminal ileum; stores last years
↑ homocysteine and ↑ MMA
B12 — nerve damage
Subacute combined degeneration: dorsal columns, lateral corticospinal tracts, spinocerebellar tracts; peripheral neuropathy; dementia.
MMA buildup → abnormal myelin fatty acids
Can occur without anemia; folate masks the anemia, not the neuropathy
Pernicious anemia
autoimmune
Antibodies to parietal cells and intrinsic factor → atrophic gastritis of body and fundus.
Achlorhydria → ↑ gastrin
Risk of gastric adenocarcinoma and carcinoid; other autoimmunity · IM B12
Orotic aciduria
rare, inherited
UMP synthase defect → pyrimidine synthesis fails.
Megaloblastic anemia that does not respond to B12 or folate; orotic acid in urine; no hyperammonemia
Treat with uridine
Drug causes
Methotrexate (DHFR), hydroxyurea, 5-FU, zidovudine, trimethoprim.
Block DNA synthesis directly
Leucovorin rescues methotrexate toxicity
Nonmegaloblastic
no hypersegs
Alcohol, liver disease, hypothyroidism, reticulocytosis, MDS, Diamond-Blackfan anemia.
Membrane lipid changes or immature/abnormal precursors
Diamond-Blackfan: infant red cell aplasia, thumb anomalies, ↑ HbF

Mechanism — two B12 enzymes

Homocysteine+ methyl-THF
→
Methionine synthaseB12 needed
→
Methionine + THFTHF → thymidine → DNA
Methylmalonyl-CoA
→
Methylmalonyl-CoA mutaseB12 needed
→
Succinyl-CoABlock → ↑ MMA → myelin damage

Without B12, folate is stuck as methyl-THF (the "folate trap"), so DNA synthesis fails even when folate levels are normal.

B12 vs folate deficiency

FEATURE
B12
FOLATE
Homocysteine
↑
↑
Methylmalonic acid
↑
Normal
Neurologic signs
Yes
No
Stores last
Years
Months
Absorbed in
Terminal ileum (with IF)
Jejunum
Classic patient
Pernicious anemia, vegan, ileal disease
Alcoholic, pregnant, methotrexate

How it's tested

COMMON CONFUSIONMacrocytosis without hypersegmented neutrophils is usually not megaloblastic — think alcohol, liver, thyroid, reticulocytosis.
THE TRAPA patient given folate for a macrocytic anemia improves on the CBC but develops ataxia and loss of vibration sense. B12 deficiency was the real cause.
YOUR APPROACHMacrocytic → hypersegs? → B12 + folate levels → MMA if unclear → find the cause (antibodies, diet, ileum, drugs).

If you see… think…

MCV 118, hypersegs, ↑ MMA, ↑ homocysteine → B12 deficiency
Same picture with normal MMA → folate deficiency
Alcoholic with poor diet, macrocytic anemia → folate deficiency
Long-term vegan with paresthesias and ataxia → B12 deficiency
Anti-intrinsic factor antibodies, ↑ gastrin → pernicious anemia
Positive Romberg + spastic legs + macrocytosis → subacute combined degeneration
Ileal resection for Crohn disease → B12 malabsorption
Raw freshwater fish eater with B12 deficiency → Diphyllobothrium latum
Megaloblastic anemia unresponsive to B12/folate + urinary orotic acid → orotic aciduria
Infant with red cell aplasia and triphalangeal thumbs → Diamond-Blackfan anemia