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MICROANGIOPATHIC HEMOLYTIC ANEMIA - MAHA.pptx
This document discusses microangiopathic hemolytic anemia (MAHA), which results from the fragmentation of red blood cells as they pass through the microcirculation. Some key causes of MAHA include disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. Laboratory findings of MAHA include decreased hemoglobin, peripheral blood smears showing fragmented red blood cells called schistocytes, and sometimes thrombocytopenia.
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An introduction to microangiopathic hemolytic anemia by Dr. N. Manjula.
Fragmentation syndrome is explained, detailing how red blood cells can undergo fragmentation due to physical trauma, leading to hemolytic anemia.
Classifies hemolytic anemia according to the site of hemolysis: macroangiopathic (large vessels) and microangiopathic (capillaries).
Describes macroangiopathic hemolytic anemia, often resulting from trauma to RBC due to abnormal vascular surfaces like heart valves.
Defines microangiopathic hemolytic anemia caused by abnormal narrowing in microcirculation, leading to turbulent blood flow.
Lists causes of microangiopathic hemolytic anemia (MAHA), including conditions like DIC and TTP which contribute to RBC trauma.
Discusses lab findings including decreased hemoglobin, fragmented RBCs, increased WBC, and thrombocytopenia.
Explains the significance of reticulocytes, immature RBCs released during hemolytic anemia in response to increased erythropoiesis.
Details the morphology of fragmented RBCs, including types like helmet cells, which result from mechanical destruction.
Describes spherocytes and their characteristics seen in immune-mediated hemolytic anemia and related conditions.
Focuses on the appearance of burr cells in blood smears indicating certain hemolytic conditions.
Explains the identification of schistocytes as a significant marker of microangiopathic hemolytic anemia.
Describes polychromatophils, their appearance, and importance in assessing anemias.
Focuses on helmet cells, their characteristics, and their role in the diagnosis of hemolytic anemia.
Explains March Hemoglobinuria, a hemolytic disorder resulting from mechanical destruction of RBCs during vigorous exercise.
Thank you slide, concluding the presentation.
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MICROANGIOPATHIC HEMOLYTIC ANEMIA - MAHA.pptx
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- 2. FRAGMENTATION SYNDROME • Redcells when subjected to physical or mechanical trauma in the circulation may undergo fragmentation. • This causes intravascular hemolysis and results in hemolytic anemia. • These are known as FRAGMENTATION SYNDROME.
- 3. CLASSIFICATION • Hemolytic anemiaresulting from trauma to RBC is classified according to the site of hemolysis: • MACROANGIOPATHIC (large vessels) • MICROANGIOPATHIC (capillaries)
- 4. MACROANGIOPATHIC HEMOLYTIC ANEMIA • Resultsmost often due to direct red cell trauma from abnormal vascular surface. • Prosthetic heart valve, synthetic vascular graft. • Known as CARDIAC HEMOLYTIC ANEMIA.
- 5. MIROANGIOPATHIC HEMOLYTIC ANEMIA • Resultsfrom abnormal luminal narrowing in microcirculation, most often due to deposition of fibrin and platelets that cause turbulent blood flow.
- 6. CAUSES OF MAHA •DISSEMINATED INTRAVASCULAR COAGULATION • THROMBOTIC THROMBOCYTOPENIC PURPURA • HEMOLYTIC UREMIC SYNDROME • MALIGNANT HYPERTENSION • SYSTEMIC LUPUS ERYTHEMATOSUS • DISSEMINATED CANCER • HELLP SYNDROME – HEMOLYSIS with ELEVATED LIVER enzymes and LOW PLATELET
- 7. LAB FINDINGS • Hb:decreased. • Mild to moderate anemia. • Peripheral smear: • RBC: fragmented RBC (SCHISTOCYTES), Polychromasia, with or without nRBC and microspherocytes. • The fragmented red cells assume different shapes like triangular cells, burr cells, helmet cells – hallmark of diagnosis of this group of hemolytic anemias. • WBC: WNL or increased. • Platelet: often thrombocytopenia is seen.
- 8. Usually refers to thepolychromatophilic RBC which is less mature non-nucleated red blood cell with a light basophilic (light blue-purple) color on peripheral smear which is due to the presence of cytoplasmic RNA. Increased numbers may be seen in certain anemias such as hemolytic anemia in which earlier RBCs are being released by the reactive bone marrow in response to the anemia with the earlier release of these less mature RBCs into circulation. Special stain can highlight the RNA molecules in these cells and on the special stain these cell are known as reticulocytes.
- 9. These are irregularlyshaped assymetrical fragmented RBCs that may have several morphologic forms (e.g. Helmet cells, etc.). It is usually the result of mechanical disruption of the RBCs and many of which are generated by getting stuck to fibrin strands within the vessel wall (intravascular destruction) which may be secondary to a Microangiopathic hemolytic anemia such as HUS, TTP and DIC or a fibrin-independent destructive path such as being broken up by a mechanical heart valve.
- 10. Small round RBCswith no central pallor that may be seen in in blood smears from patients with immune- mediated hemolytic anemia, burn injuries, and hereditary spherocytosis.
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- 15. MARCH HEMOGLOBINURIA • Thishemolytic disorder develops in athelets and soldiers following long distance running or marching, prolonged walking and vigorous exercise. • Mechanical destruction of red cells occurs in the capillaries of the feet and results in transient hemoglobinemia and hemoglobinuria.
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