![GENERAL FEATURES
OF HEMOLYTIC DISORDERS
GENERAL EXAMINATION - JAUNDICE, PALLOR
BOSSING OF SKULL
PHYSICAL FINDINGS - ENLARGED SPLEEN
HEMOGLOBIN - FROM NORMAL TO SEVERELY REDUCED
MCV - USUALLY INCREASED
RETICULOCYTES - INCREASED
BILIRUBIN - INCREASED[MOSTLY UNCONJUGATED]
LDH - INCREASED
HAPTOGLOBULIN - REDUCED TO ABSENT](https://image.slidesharecdn.com/sesu-161210151447/75/lab-work-up-for-hemolytic-anemia-3-2048.jpg)
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lab work up for hemolytic anemia
This document discusses the workup and evaluation of hemolytic anemia. Common tests include a complete blood count, peripheral smear, LDH, haptoglobin, and indirect bilirubin. Changes in LDH and low haptoglobin are sensitive indicators of hemolysis. Hemolytic anemias can be hereditary or acquired. The workup depends on the suspected cause and may include hemoglobin electrophoresis, Coombs testing, and flow cytometry to identify the specific type of hemolytic anemia.
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lab work up for hemolytic anemia
- 1. LAB WORKUP FOR HEMOLYTICANAEMIA
- 2. Common investigation •Complete bloodcell count •Peripheral blood smear •Serum lactate dehydrogenase (LDH) study •Serum haptoglobin •Indirect bilirubin •Changes in the LDH and serum haptoglobin levels are the most sensitive general tests because the indirect bilirubin is not always increased.
- 3. GENERAL FEATURES OF HEMOLYTICDISORDERS GENERAL EXAMINATION - JAUNDICE, PALLOR BOSSING OF SKULL PHYSICAL FINDINGS - ENLARGED SPLEEN HEMOGLOBIN - FROM NORMAL TO SEVERELY REDUCED MCV - USUALLY INCREASED RETICULOCYTES - INCREASED BILIRUBIN - INCREASED[MOSTLY UNCONJUGATED] LDH - INCREASED HAPTOGLOBULIN - REDUCED TO ABSENT
- 4. RBC: Increased reticulocyte count. WBC:neutrophilia PLATELETS: Thrombocytosis CELLULARITY: Increased ERYTHROPOIESIS: increased normoblasts M:E RATIO : decreased PERIPHERAL SMEAR BONE MARROW
- 5. CLASSIFICATION OF HEMOLYTICANEMIA Intra corpuscular Extra corpuscular Hereditary Hemoglobinopathies, Enzym0pathies, Red cell membrane defect. Familial haemolytic uremic syndrome. Acquired Paroxysmal nocturnal haemoglobinuria Auto immune, Mechanical trauma, Toxic agents, Drugs, Infections.
- 6. LABORATORY EVALUATION OFHEMOLYSIS
- 7. HEREDITARY • CBC • Hemoglobinopathyevaluation • DNA analysis—not routinely done but can be used to help diagnose hemoglobin variants, thalassemia, and to determine carrier status • G6PD test—to detect deficiency in this enzyme • Osmotic fragility test—detects RBCs that are more fragile than normal, which may be found in hereditary spherocytosis
- 8. ACQUIRED • Tests forautoantibodies for suspected autoimmune disorders • Direct antiglobulin test (DAT) in the case of transfusion reaction, mother- baby blood type incompatibility, or autoimmune hemolytic anemia • Haptoglobin—usually low • Reticulocyte count—typically high • Flow cytometry for suspected PNH
- 9. SICKLE CELL ANEMIA PERIPHERALBLOOD SERUM FINDING PERIPHERAL SMEAR BONE MARROW HB: REDUCED. ESR & PCV: REDUCED RETICULOCYTE COUNT: INCREASED BILIRUBIN :Increased IRON STATUS : increased SERUM IRON , TRANSFERRIN , FERRITIN HAPTOGLOBIN: decreased URINE UROBILINOGEN: INCREASED RBC:NORMOCYTIC,NORMOCHROMIC, ANISOPOIKILOCYTOSIS,SICKLE CELL ,POLYCHROMATOPHILIA WBC: MIDLY INCREASED PLATELETS: MIDLY INCREASED CELLULARITY: Increased ERYTHROPOIESIS: normoblastic erythroid hyperplasia Iron stores : increased
- 10. Diagnosis • Sickling test •Hb electrophoresis • Estimation of HBF • Solubility test for HBs • Confirmatory test : HPLC (High Performance Liquid Chromatography) • Prenatal genetic testing
- 11.
- 12. BONE MARROW :- •CELLULARITY : Hypercellular • M:E RATIO : 1:1 to 1:5 • ERYTHROPOIESIS : normoblastic with erythroid hyperplasia • BONE MARROW IRON : increased Biochemical findings : • BILIRUBIN : Increased • URINE UROBILINOGEN: Increased • SERUM HAPTOGLOBIN : Reduced • SERUM IRON STATUS: serum iron , ferritin , transferrin increased • TOTAL IRON BINDING CAPACITY : reduced Special test :- • HbF : increased • HB electrophoresis • HPLC : confirmatory test • ESTIMATION OF GLOBIN CHAINS • HBA2 • NESTROF TEST(Naked eye single tube osmotic fragility test) : POSITIVE for beta thalassemia minor.
- 13. PERIPHERAL BLOOD BIOCHEMICALFINDING PERIPHERAL SMEAR SPECIAL TEST HB: REDUCED. MCV: REDUCED MCHC: INCREASED & >35 g/dl RETICULOCYTE COUNT : Increased BILIRUBIN :Increased HAPTOGLOBIN: decreased URINE UROBILINOGEN: INCREASED RBC: spherocytes are distinctive – small and dark stained . Rbc has no central pallor. Polychromatophilia. WBC: TLC increased PLATELETS: normal AUTO-HEMOLYSISTEST: shows spontaneous hemolysis DIRECT ANTIGLOBULIN(COOMB’S) TEST: NEGATIVE OSMOTIC FRAGILITYTEST: increased Hereditary spherocytosis
- 14. GLUCOSE 6-PHOSPHATE DEHYDROGENASE DEFICIENCY PERIPHERALBLOOD : HB : decreased RETICULOCYTE : increased SELF – LIMITED HEMOLYSIS : old cells are hemolyzed URINE :hemoglobinuria RBC ENZYME ANALYSIS : positive PERIPHERAL SMEAR : RBC : anisopoikilocytisis with polychromatophilia , microspherocytes and bite cells . Heinz bodies WBC: mild leukocytosis PLATELETS : normal
- 15. Immunohemolytic anemia PERIPHERAL BLOOD: HB : decreased RETICULOCYTE : increased ANTIGLOBULIN TEST (Coombs test ) : antibodies in the mother and baby are detected by direct and indirect coombs test SERUM BILIRUBIN : increased LDH : increased HAPTOGLOBIN : decreased PERIPHERAL SMEAR : RBC : normocytic normochromic anemia with nucleated RBC , polychromatophils WBC: normal . PLATELETS : normal
- 16. PAROXYSMAL NOCTURNAL HEMOGLOBINURIA •HAM’S ACIDIFIED SERUM TEST AND DUCROUS HEMOLYSIS TEST • FLOW CYTOMETRY