Megaloblastic anemia
Megaloblastic anemia is an anemia that results from inhibition of DNA synthesis during red blood cell production. When DNA synthesis is impaired, the cell cycle cannot progress from the G2 growth stage to the mitosis stage. This leads to continuing cell growth without division, which presents as macrocytosis.
Megaloblastic anemia has a rather slow onset, especially when compared to that of other anemias.
The defect in red cell DNA synthesis is most often due to hypovitaminosis, specifically vitamin B12 deficiency or folate deficiency. Loss of micronutrients may also be a cause. Copper deficiency resulting from an excess of zinc from unusually high oral consumption of zinc-containing denture-fixation creams has been found to be a cause.
Megaloblastic anemia not due to hypovitaminosis may be caused by antimetabolites that poison DNA production directly, such as some chemotherapeutic or antimicrobial agents.
The pathological state of megaloblastosis is characterized by many large immature and dysfunctional red blood cells in the bone marrow and also by hypersegmented neutrophils. These hypersegmented neutrophils can be detected in the peripheral blood .
Causes
- Vitamin B12 deficiency:
- * Achlorhydria-induced malabsorption
- * Deficient intake
- * Deficient intrinsic factor, a molecule produced by cells in the stomach that is required for B12 absorption
- * Coeliac disease
- * Biological competition for vitamin B12 by diverticulosis, fistula, intestinal anastomosis, or infection by the marine parasite Diphyllobothrium latum
- * Selective vitamin B12 malabsorption
- * Chronic pancreatitis
- * Ileal resection and bypass
- * Nitrous oxide anesthesia.
- Folate deficiency:
- * Alcoholism
- * Deficient intake
- * Increased needs: pregnancy, infant, rapid cellular proliferation, and cirrhosis
- * Malabsorption
- * Intestinal and jejunal resection
- * Deficient thiamine and factors responsible for abnormal folate metabolism.
- Combined Deficiency: vitamin B12 & folate.
- Inherited Pyrimidine Synthesis Disorders: Orotic aciduria
- Inherited DNA Synthesis Disorders
- Toxins and Drugs:
- *Folic acid antagonists
- * Purine synthesis antagonists
- * Pyrimidine antagonists
- * Phenytoin
- * Nitrous Oxide
- Erythroleukemia
- Inborn genetic mutations of the Methionine synthase gene
- Di Guglielmo's syndrome
- Congenital dyserythropoietic anemia
Pathophysiology
In the bone marrow, most megaloblasts are destroyed prior to entering the peripheral blood some can escape the bone marrow to peripheral blood but they are destroyed by the reticulo-endothelial system.
Diagnosis
The gold standard for the diagnosis of Vitamin B deficiency is a low blood level of Vitamin B. A low level of blood Vitamin B is a finding that normally can and should be treated by injections, supplementation, or dietary or lifestyle advice, but it is not a diagnosis. Hypovitaminosis B can result from a number of mechanisms, including those listed above. For determination of cause, further patient history, testing, and empirical therapy may be clinically indicated.A measurement of methylmalonic acid can provide an indirect method for partially differentiating Vitamin B and folate deficiencies. The level of methylmalonic acid is not elevated in folic acid deficiency. Direct measurement of blood cobalamin remains the gold standard because the test for elevated methylmalonic acid is not specific enough. Vitamin B is one necessary prosthetic group to the enzyme methylmalonyl-coenzyme A mutase. Vitamin B deficiency is but one among the conditions that can lead to dysfunction of this enzyme and a buildup of its substrate, methylmalonic acid, the elevated level of which can be detected in the urine and blood.
Due to the lack of available radioactive Vitamin B, the Schilling test is now largely a historical artifact. The Schilling test was performed in the past to help determine the nature of the vitamin B deficiency. An advantage of the Schilling test was that it often included Vitamin B with intrinsic factor.
Blood findings
The blood film can point towards vitamin deficiency:- Decreased red blood cell count and hemoglobin levels
- Increased mean corpuscular volume and mean corpuscular hemoglobin
- Normal mean corpuscular hemoglobin concentration
- Decreased reticulocyte count due to destruction of fragile and abnormal megaloblastic erythroid precursor.
- The platelet count may be reduced.
- Neutrophil granulocytes may show multisegmented nuclei. This is thought to be due to decreased production and a compensatory prolonged lifespan for circulating neutrophils, which increase numbers of nuclear segments with age.
- Anisocytosis and poikilocytosis.
- Macrocytes are present.
- Ovalocytes are present.
- Howell-Jolly bodies also present.
- An increased lactic acid dehydrogenase level. The isozyme is LDH-2 which is typical of the serum and hematopoetic cells.
- Increased homocysteine and methylmalonic acid in Vitamin B deficiency
- Increased homocysteine in folate deficiency
Bone marrow shows megaloblastic hyperplasia.