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2001
hematology
| Question | Answer |
|---|---|
| HbF hemoglobin F | |
| hemoglobin H HbH | tetramer of beta-globin chains |
| 1st stage of Iron deficiency | depleted iron stores no anemia yet normocytic and normochromic RBC |
| Alpha-thallasimeia | deletion of alpha-globin genes |
| Intrinsic factor | Pernicious anemia |
| Vitamin B12 deficiency | megaloblastic anemia |
| Pancytopenia | low numbers of all cell types |
| Transcobalamin | transfers B12 to tissues |
| hypersegmented neutrophils | Megaloblastic anemia |
| Vitamin B12 | co-factor for the synthesis of dUMP to dTMP |
| Macrocytic anemia | MCV>100fL |
| Megaloblastic anemia causes | B12 deficiency and folate deficiency |
| Normoblastic anemia | normoblastic maturations |
| normal retic count, Howell-Jolly bodies, | megaloblastic anemia- Vitamin B12 or folate low levels |
| Normal retic count, no Macro-ovalocytes, Dacryocytes, Howel-Jolly bodies, or Hypersegmented Neutrophils | Round macrocytes----normoblastic anemia |
| normoblastic anemia causes | alcoholism, liver disease aplastic anemia, hemolytic anemia with riticulocytosis |
| Vitamin B12 deficiency symptoms | Glossitis-beefy red tongue, smooth pale tongue |
| MCV 100-140 fL, MCH raised, MCHC normal, retic count normal | Macrocytic normochromic anemia |
| hypersegmented neutrophils | > 5 lobes in the nucleus |
| Methylmalonic acid (MMA) | normal in folate deficiency elevated in B12 deficiency |
| Homocysteine levels | elevatd in both Vit B12 and Folate deficiency |
| folate needed for | Histidine to glutamicacid. Methionine sythesis. Thymidine synthesis (dUMP-dTMP) |
| Folic acid absorbed | in proximal Jejunum |
| Folic acid in food | reduced to N5-methyl-THF |
| demethylating N5-methyl-THF | Vitamin B12 needed |
| Folic acid requirements | 50ug a day needed liver stores 3-6 month supply |
| B12 transport | TCI, TCII, (primary plasma protien for B 12 transfer, & TCIII |
| B12 stores | normally about 5,000ug last about 1000 days. 3-5 ug needed per day |
| Anti-Intrinsic factor antibodies | Pernicious anemia |
| Schilling test | oral administration of radioactive B12, 24 urine assayed for radioactivity. repeat 5 days later with radioactive B12 complexed to intrinsic factor. if secound is normal Dx is Pernicious anemia, if abnormal Dx is malabsobrtion. |
| infestation with Diphyllothrium latum | Vitamin B12 deficiency |
| CDA I | Autosomal recessive disorder, Megaloblastic erythroblasts, binucleated NRBC. |
| CDA II | Autosomal recessive disorder, RBC not megaloblastic, multinucleated up to 7 nuclei. HEMPAS most common |
| CDA III | Autosomal dominant disorder Giant Erythroblasts, up to 16 nuclei |
| Megaloblastic anemia | is a marcocytic anemia with defective nuclear maturation caused by DNA synthesis imparment |
| Megaloblastic anemia causes | Vitamin B12 deficiency and or folate deficiency |
| vitamin B12 deficiency | main cause is pernicious anemia |
| Folic acid deficiency | main cause is poor diet |
| Macrocytic nonmegaloblastic anemia | main cause is alcoholism and liver disease |
| INCREASED RETICS | INCREASED BONE MARROW ACTIVITY |
| RBC LIFE SPAN | 100-120 DAYS |
| EXTRAVASCULAR SITES OF DESTRUCTION | PHAGOCYTES IN TISSUES(MOST COMMON TYPE) PHAGOCYTES IN SPLEEN LIVER, OR BONE MARROW SPLENIC CORDS |
| INTRINSIC RBC DEFECTS | MEMBRANE, ENZYMES DEFECTS MOST ARE HEREDITARY |
| EXTRINSIC RBC DEFECTS | TOXINS IN PLASMA, ANTIBODIES AGAINST RBCS, PHYSICAL OR MECHANICAL TRAUMA. USUALLY AQUIRED |
| hemoglobin S | point mutation at 6th postion Glutamic acid to Valine (beta chain) |
| Hemoglobin C | point mutation at 6th postion Glutamic acid to Lysine (beta chain) |
| Sickle cell anemia | HbSS most common type of sickle cell. person is homozygous for the HbS gene mutation |
| three types of crises with sickle cell disease | aplastic anemia, hemolytic anemia, and vaso-occlusive (painful) |
| Sickle cell disease | no HbA present, 80% HbS, rest HbA2 and HbF |
| Sickle cell trait | HbA and HbS present in a 60:40 ratio |
| hemoglobin SC disease | when heterozygous HbS and HbC gene mutations are present |
| HbS definitive test | electrophoresis on cellulose acetate and citrate agar |
| clinical laboratory findings in infectious mononucleosis | luekocytosis, lymphocytosis, >20% reactive lymphocytes, heterophil antibodies, positive antigen test for EBV |
| nonmalignant conditions with lymphocytosis | infectious mononucleosis, infectious lymphocytosis, Bordatella pertussis infection, cytomegalovirus infection, Toxoplasmosis,persistant polycolonal B cell lymphocytosis, Viral infections, Chronic infections |
| nonmalignant conditions with lymphocytosis -2 | endocrine disorders, convalescence of acute infections, immune reactions, inflammatory diseases |
| Malignant conditions with lymphocytosis | Acute lymphocytic leukemia, Chronic lymphocytic leukemia, hairy cell leukemia, heavy chain disease, multiple myeloma, Waldenstroms macroglobuliemia. |
| conditions with lymphocytopenia | malnutrition, disseminated neoplasms, connective tissue disease, Hodgin's disease, chemotherapy, radiotherapy, corticosteroids, acute inflammatory disease, acquired immune disease, renal disease, stress |
| SCIDS | decreased: IgG,IgM, IgA, B-lymphocytes, absent mature T lymphocytes. |
| Wiskott-Aldrich syndrome | Decreased: IgM, T lymphocytes(progressive) Increased: IgA, IgG. B lymphocytes normal |
| DiGeorge syndrome | Decreased: T lymphocytes. all else normal |
| X-linked agammaglobulimemia | Decrease: IgG,IgM, IgA, B lymphocytes. T lymphocytes normal |
| Hereditary ataxia-telangiectasia | Decreased: IgG, IgA, IgE, T lymphocytes. Up: IgM. B lymphocytes normal. |
| conditions with Neutrophilla | Acute bacterial & fungal infections, burns, trauma, surgery, other inflammatory processes, metabolic alterations, Neoplasma, acute hemmorage, rebound from BMT, certain toxins, drugs,and chemicals, physiologic neutrophilla, chronic myeloprolifeative disord |
| leukocyte count - leukemoid reaction | increased up to > 50 X 10^9/L |
| Leukocyte count - CML | marketly increased usually > 50 X 10^9/L |
| Leukemia | primarily BM and blood involvement, myeloid or lymphoid origin, secondary involvement of lymph tissues |
| Lymphoma | primarily lymph node or solid tissues involvement, lymphoid origin, secondary involvement of BM and Blood. |
| myeloprolifertive disorders | myelodysplastic syndromes, chronic myeloprolifertive syndrome, acute myeloid leukemias. |
| lymphoprolifertive disorders | acute lymphoblastic leukemia, chronic lymphocytic leukemia, non-Hodgkin lymphomas, Hodgin's disease |
| Chronic leukemias ?blasts | <20% blasts leukocytosis, all stages of maturation. predominance of mature cells |
| Acute Leukemias | many blasts >20% some mature forms |
| Acute Leukemias | age-all ages clinical onset - sudden course of disease - weeks to months predominant cell - blasts, some mature forms anemia - mild-severe thrombocytopenia - mild-severe WBC - variable |
| Chonic Leukemias | age - adults clinical onset - insidious course of disease - months to years predominant cell - mature forms anemia - mild throbocytopenia - mild WBC - increased |
| PAS stain | stains carbohydrates, myleoblasts neg lymphoblasts chunky positive M6 erthroblasts chunky positive |
| MPO stain | positive - myeloblasts negative - lymphoblasts,monoblasts,erythrobalsts. |
| SBB stains (Sudan Black) | stains lipids same patterns as MPO stain |
| CAE stains | positive in myeloblasts negative in monoblasts & lymphoblasts |
| NSE stain | negative in myeloblasts & lymphoblasts positive in monoblasts |
| LAP stain (leukocyte alkaline phosphatase) | present in neutrophil granules not present in eosinophils, or basophils |
| Acid phosphotase | present in lysosomes. t-cell ALL focal polarized activity. |
| terminal deoxnucleotidyl transferase (TdT) | nuclear DNA Polymerase. present in 90-95% off ALL cases (T & B cell) |
| Toluidine blue | specifically for Basophils and mast cells (+) |
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