Term
| What are the three forms of adult hemoglobin and which one is most common? |
|
Definition
1) HbA (96%) - most common
2) HbA2 (2-3%)
3) HbF (<2%) |
|
|
Term
| How is the severity of anemia determined in general? |
|
Definition
1) rate of RBC destruction
2) rate of erythropoesis |
|
|
Term
| What are most forms of hemolysis? |
|
Definition
| extravascular: removed by reticuloendothelial system |
|
|
Term
| What is used to confirm the diagnosis of hereditary spherocytosis? |
|
Definition
| 24-hour incubated osmotic fragility test |
|
|
Term
| What are the 3 classical presentations of paroxysmal nocturnal hemoglobinuria (PNH) |
|
Definition
1) acute intravascular hemolysis superimposed on chronic hemolysis
2) hemoglobinuria after sleep
3) recurrent venous occlusions leading to embolisms and vein thrombosis |
|
|
Term
| What is the most common hereditary enzymopathy? |
|
Definition
| Glucose-6-Phosphate dehydrogenase defieciency |
|
|
Term
| Who is at more risk for G6PD? |
|
Definition
|
|
Term
| What 2 unique findings are on a blood smear for G6PD deficiency? |
|
Definition
1) bite cells
2) Heinz bodies |
|
|
Term
| What is the molecular abnormality associated with Sickle cell disease |
|
Definition
| substitution of valine for glutamine at the 6th position of the beta-hemoglobin chain- HbS |
|
|
Term
| Sickle cell trait is characterized by what percentage of HbS? |
|
Definition
|
|
Term
| What are the two major clinical findings associated with sickle cell disease? |
|
Definition
1) Chronic hemolysis
2) Acute, episodic vaso-occlusive crisis |
|
|
Term
| Localized renal ischemic changes and loss of urine concentrating abilities are due to? |
|
Definition
| sickling occurring in renal medulla with sickle cell disease |
|
|
Term
| What are the 4 risk factors for a painful crisis in sickle cell disease? |
|
Definition
1) Hemoglobin level >8.5 g/dl
2) Pregnancy
3) Cold weather
4) High reticulocyte count |
|
|
Term
| blockage from thrombosis in sickle cell disease is most commonly found where? |
|
Definition
|
|
Term
| What is the definitive test for sickle cell disease? |
|
Definition
| hemoglobin electrophoresis |
|
|
Term
| What is a skeletal complication associated with sickle cell disease? |
|
Definition
| aseptic necrosis of femoral head |
|
|
Term
| Cardio problems with sickle disease are? |
|
Definition
| Acute MIs without coronary disease |
|
|
Term
| What is the major cause of morbidity and mortality in sickle cell disease? |
|
Definition
| pneumonia with chlamydia pneumoniae and mycoplasma pneumoniae |
|
|
Term
| What is a hepatobiliary complication of sickle cell disease? |
|
Definition
|
|
Term
| What dermatological complications are seen with sickle cell disease? |
|
Definition
|
|
Term
| What are the 3 variants of sickle cell disease? |
|
Definition
1) sickle trait
2) sickle cell-B-thalassemia
3) sickle cell-hemoglobin C disease |
|
|
Term
| What are methemoglobinemia most commonly secondary to? |
|
Definition
| ingestion of oxidizing agents that overwhelm the system |
|
|
Term
| What globin chains are diminished and which ones are in excess with B-thalasemias? |
|
Definition
1) diminshed b-globin chains
2) excess a-chains
*a-thalasemias are opposite |
|
|
Term
| thalassemias are suspected if what two things are seen? |
|
Definition
1) MCV <75 fl
2) RBC >5 million cells /ul |
|
|
Term
| What is b-thalassemia characterized by? |
|
Definition
| severe anemia in the 1st year of life |
|
|
Term
| What are the 3 presentations of a-thalassemias |
|
Definition
1) Hemoglobin Barts
2) Hemoglobin H disease
3) Heterozygous a-thalassemia |
|
|
Term
| Warm/cold antibodies are usually what? |
|
Definition
|
|
Term
| Autoimmune hemolytic anemia is usually what kind of hemolysis? |
|
Definition
|
|
Term
| Drug related hemolysis is often indistinguishable from what? |
|
Definition
| autoimmune hemolytic anemia |
|
|
Term
| Coombs testing shows what with Drug-related immune hemolysis? |
|
Definition
| anti-IgG reagent positive but negative with anti-C3d |
|
|
Term
| What is the classic drug associated with alteration of a membrane antigen? |
|
Definition
|
|
Term
| Cold agglutinin is seen with what in young adults and what with 60 year old patients |
|
Definition
young adults-infections (mycoplasma pneumoniae or infections mononucleosis)
60 y/0- prodome to a lympoproliferative or immunoproliferative disorder |
|
|
Term
| Coomb test is positive for what in Cold agglutinin? |
|
Definition
|
|
Term
| Treatment for metheoglobinemia? |
|
Definition
|
|
Term
| What are 2 classical examples of enzymatic attack on RBCs? |
|
Definition
1) snake-venom
2) clostridial lecithinases |
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