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61 Special Hematology BONE MARROW PREPS HEMOGLOBIN ELECTROPHORESIS...

61 Special Hematology BONE MARROW PREPS HEMOGLOBIN ELECTROPHORESIS 1. Reference ranges 1. Cellulose acetate, pH 8.6; from cathode a. Megakaryocytes - 5/lpf to anode (- to +) h. Myeloid: erythroid ratio - 3:1 - 4:1 Origin + 2. Clinical correlations I I I I a. ""Dr y" tap - aplastic anemia, s F A A2 myelofibrosis C D b. tM:E ratio - erythroid hyperpla- E G sia, hemolytic anemia, ery- 0Arab Lepore throleukemia CHartem c. M:E ratio - myeloid hyperplasia, myeloid leukemias REMEMBER! Hemoglobin Migration (pH 8.6) REMEMBER! C (crawl), S (slow), f (fast), and A (accelerate) Bone Marrow 2. Citrate Agar, pH 6.2 Origin + M:E Ratio 3:1 - 4:1 A Aspirate - sternum & hip (iliac crest) I I I I most common - for morphology review F A C s R Romanowsky Stains (Most other hemoglobins migrate with A at pH 6.2) R Ratio of Cells:Fat (50% cells:50% fat) 0 Overall Evaluation of Cellularity - use biopsy WBC Differential (determine type of '----_.J leukemia) Red Cell Disease States HEMOGLOBINOPATHIES a. H emoglobin S 1. Structural mutations (most common ❖ Heterozygous - asymptomatic involve ~-chain) (sickle cell trait) ❖ Homozygous - severe chronic HEMOGLOBIN COMMENTS hemolytic anemia with many complications (sickle cell disease) s Valine for Glutamic Acid ❖ See sickle cells on Wright (not in (6th Position, Beta Chain) trait) ❖ Sickle Dex - reducing agent (Na C Lysine for Glutamic Acid (6th Position, Beta Chain) dithionite) causes Hh S to precipitate producing turbidity D East Indian Individuals, Migrates with HbS at 8.6 ❖ Confirmed by electrophoresis e- Cellulose acetate - pH 8.6 E Southeast Asian Individuals, Migrates with Hb C (migrates with D, G, Lepore) and A2 at 8.6 (hypochromic, microcytic) ,g, Citrate agar - pH 6.2 (S separates from othen) 62 b. Hemoglobin C i& Chronic hemolytic disease ❖ Heterozygous - asymptomatic ~ Hb H (~4 ) ❖ Homozygous - mild chr011ic i& Hb Bar t's (Y4) present at birth anen11a w Hb H inclusions (h eiuz bodies) ❖ May see Hh C crystals (bar- ❖ 4 deleted a genes (- -I- -) shaped), and target cells w H y drops fetalis ❖ In SC disease, crystals appear as i& Non viable fetus "hand in glove" or "Washington monument" ❖ Confirm by electrophoresis ~ Cellulose a cetate - migrates with A2, E, 0 , and CHarlem ~ Citrate agar - (C separates @ Sexy REMEMBER! from others) 2. Decrea sed production of a or ~ chains Valine a. ~ thalassemia - t or absent produ ction of ~-ch ain s ❖ Microcytic, hypochromic anemia ❖ ,t. Hb Az and F, t or absent A t b. a thalasserma - production of Valine for Glutanuc Acid in Hemoglobin S a-chains ❖ 1 deleted a gene (- a/aa) - Silent carrier; n ormal CBC ❖ 2 deleted a genes (- al-a) or (- -laa) - Mild microcytic, hypochromic anemia ❖ 3 deleted a genes (- - I-a) - Hemoglobin Variants BD.d Hemoglobin H disease Electrophoretic Migrations Classification of Anemias Anemia--- "t Hb & Hct - MCV Normal (normocytic) PROBLEM WITH HEME * ANTIBODY DESTRUCTION MEGALOBLASTIC Iron Deficiency Hemolytic Disease of the Newborn MATURATION Sideroblastic Transfusion Reaction B12 Deficiency Chronic Disease/Inflammation Autoimmune Hemolytic Anemia Folate Deficiency Myeloproliferative Neoplasms PROBLEM WITH GLOBIN RBC MEMBRANE DEFECT Myelodysplastic Syndromes Thalassemias Hereditary Spherocytosis (HS) NON-MEGALOBLASTIC Hemoglobin E Hereditary Elliptocytosis (HE) MATURATION Paroxysmal Nocturnal Hemoglobinuria(PNH) ENZYME DEFICIENCY Liver Disease G6PD Pyruvate Kinase (PK) tPRODUCTION, LOSS Aplastic Anemia, Chronic Renal Disease Acute Blood Loss, BM infiltration HEMOGLOBINOPAT HIES Hb S, HbC OTHER EXTERNAL ACTIONS Physical Trauma, Chemicals, Organisms