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Hematology 1
| Question | Answer | |
|---|---|---|
| What are some causes of low MCV anemia? | 1. Iron deficiency 2. Thalassemia 3. Sideroblastic anemia 4. Anemia of chronic disease | |
| What are general points about microcytic anemia? | 1. Most causes of microcytic anemia is due to "production" problem. 2. That gives a low reticulocyte count 3. Except for alpha thalassemia with 3 gene deletion (high reticulocyte count) | |
| What are causes of macrocytic anemia? | 1. B12 and folate deficiency 2. Alcoholism 3. Meds (azathioprine, 6-MP, hydroxyurea, zidovudine, phenytoin) 4. Liver disease 5. Hypothyroidism 6. Myelodysplastic syndrome | |
| Is sideroblastic anemia microcytic or macrocytic? | BOTH | |
| Macrocytic anemias have high or low reticulocyte count? | LOW | |
| What are some causes of normocytic anemia? | Acute blood loss or hemolysis causes drop in hematocrit, no time for mcv to change. But eventually HIGH retic count. | |
| What is PRBC's when is it used? | 1. Concentrated form of blood 2. Unit of blood with plasma removed. 3. HCT is 70-80% 4. Each unit of PRBC will raise HCT by 3 points per unit | |
| What is FFP? | 1. FFP replaces clotting factors in those with elevated PT, aPTT, BT or INR 2. FFP is used as replacement with plasmapheresis. | |
| What is cryoprecipitate? | 1.Used to replace fibrinogen 2. Used in DIC 3. Provides high amounts of clotting factors in a smaller plasma volume 4.Never used FIRST for anything | |
| What are the general characteristics for Iron deficiency anemia? | 1. caused by blood loss 2. Body needs 1-2 mg/day. Mestruating women need 2-3/day. Pregnant women need 5-6/day. 3. Iron absorbed in duodenum 4mg/day. | |
| What are the general characteristics for anemia of chronic disease? | 1. Includes any form of cancer, chronic infection 2. Iron is locked in storage, trapped in macrophages or in ferritin. 3. Hb synthesis cannot move forward. 4. In renal failure, anemia of chronic dx is due to EPO deficiency | |
| When is sideroblastic anemia, macrocytic? | Can be macrocytic when associated with myelodysplasia. | |
| What are the general characteristics for thalassemia? | 1. Common cause of microcytosis. 2. Pts with thalassemia trait alone are asx | |
| If a pt has Blood loss (GI bleeding) what is the most likely diagnosis of anemia? | Iron deficiency | |
| If a pt has menstruation what is the most likely diagnosis of anemia? | Iron deficiency | |
| If a pt has cancer or chronic infection what is the most likely diagnosis of anemia? | Anemia of chronic disease | |
| If a pt has RA what is the most likely diagnosis of anemia? | Anemia of chronic disease | |
| If a pt is alcoholic what is the most likely diagnosis of anemia? | Sideroblastic | |
| If a pt has asx what is the most likely diagnosis of anemia? | Thalassemia | |
| What is the diagnosis if iron studies show low ferritin? | Iron deficiency | |
| What is the diagnosis if iron studies show high iron? | Sideroblastic anemia | |
| What is the diagnosis if iron studies show normal iron studies? | Thalassemia | |
| What do you look for in iron studies for iron deficiency anemia? | 1. Low ferritin is specific for iron deficiency (pts can still have normal or increased ferritin with active infection or inflammation) 2. Increased TIBC. 3. Increased RDW. 4. Low serum iron levels. | 6. Serum iron levels are low in chronic disease and iron deficiency |
| What do you look for in iron studies for chronic disease anemia? | 1. Serum Iron is low in circulation, because iron is trapped in storage. 2. So Ferritin or stored iron is high. 3. TIBC is decreased. | |
| What do you look for in iron studies for sideroblastic anemia? | 1. Only form of microcytic anemia where circulating iron is increased. | |
| What do you look for in iron studies for thalassemia anemia? | 1. Genetic disease with normal iron studies | |
| What is the most accurate test for iron deficiency? | Bone marrow biopsy. (Never done) | |
| What is the most accurate test for sideroblastic anemia? | 1. Prussian blue staining for ringed sideroblasts. 2. Basophilic stippling can occur in any cause of sideroblastic anemia | |
| What is the most accurate test for thalassemia? | 1. Hb electrophoresis 2. For alpha thalassemia- genetic studies are most accurate test. | |
| What are the electrophoresis findings for alpha thalassemia with one gene, two gene, three gene, four gene deletion? | 1. One gene deletion: Normal 2. Two gene deletion: mild anemia, normal electrophoresis 3. three gene deletion: moderate anemia with HbH, with are beta 4 tetrads, increased reticulocytes. | 4. Four gene deletion: gamma-4 tetrads or Hb Bart, CHF causes death in utero. |
| What are the the electrophoresis finding for beta thalassemia with one gene deletion and three gene deletion? | 1. One gene deletion: Increased Hb F and A2 2. Three gene deletion: beta thalassemia intermediatia- normal HbF, no transfusion dependence | |
| What is the treatment for iron deficiency? | 1. Replace with oral ferrous sulfate. 2. If insufficient then tx with intramuscular iron. | |
| What is the treatment for chronic disease? | 1. Correct the underlying disease. 2. Only anemia associated with end-stage renal failure routinely responds to EPO replacement | |
| What is the treatment for sideroblastic anemia? | 1. Correct the cause. 2. Some pts respond to vitamin B6 or pyridoxine replacement. | |
| What is the treatment for thalassemia? | 1. Trait is not treated. 2. Beta thalassemia major (Cooley anemia) is managed with chronic transfusion lifelong. 3. Iron overload is managed with deferasirox-oral iron chelator 4. Deferoxamine is parenteral version of iron chelator. | |
| What are the causes of Vit B12 deficiency? | 1. Pernicious anemia 2. Pancreatic insufficiency 3. Dietary insufficiency 4. Crohn's 5. Blind loop syndrome 6. D.latum | |
| What are the causes of folate deficiency? | 1. Dietary deficiency (goat's milk has not folate, limited iron and B12) 2. Psoriasis and skin loss or turnover 3. Drugs- phenytoin, sulfa, MTX. | |
| What is megaloblastic anemia? | 1. Presence of hypersegmented neutrophils 2. Only B12, folate def and antimetabolite med cause hypersegmentation. 3. Alcohol can give macrocytic anemia and neurological problems, it will not give hypersegmented neutrophils. | |
| What causes both B12 and folate def? | Celiac disease | |
| How does B12 deficiency present? | 1. Presents with alcoholic with peripheral neurological abnormality. 2. Posterior column damage to position and vibratory sensation is classic. 3. Look for ataxia | |
| What are the lab test findings for B12 deficiency and folate deficiency? | 1. Megaloblastic anemia 2. increased LDH 3. increased indirect bilirubin 4. Decreased reticulocyte count 5. Hypercellular bone marrow 6. Macroovalocytes 7. increased homocysteine levels. | |
| How do you confirm B12 deficiency? | 1. B12 deficiency is associated with increased Methylmalonic acid levels. | |
| What is the treatment for B12 deficiency? | Replacement. Folate will correct hematological problem, but not the neuro. | |
| What is a complication of B12 or folate replacement? | Hypokalemia. Observe and replace. | |
| What are the hemolytic anemias? (9) | 1. Sickle cell disease 2. Sickle cell trait 3. Hereditary Spherocytosis 4. Autoimmune (Warm or IgG) hemolysis 5. Cold Agglutinin disease 6. G6PD 7. HUS and TTP 8. PNH 9. Aplastic Anemia | |
| All forms of hemolytic anemia lead to? (8) | 1. Sudden decrease in HCT 2. Increased LDH, 3. Increased indirect bilirubin 4. Increased reticulocytes 5. Decreased haptoglobin level 6. Slight increase in MCV because reticulocytes are larger than normal cells 7. Hyperkalemia from cell breakdown | 8. Folate deficiency- folate stores are limited and increased production of cells use them up. |
| Chronic hemolysis is associated with? | Bilirubin and gallstones. | |
| What is sickle cell disease? | 1. Point mutation at position 6 of the beta globin chain, valine replaces glutamic acid. 2. Chronic, well compensated hemolytic anemia with a reticulocyte count that is always high. | |
| What causes an acute painful crisis in sickle cell disease? | 1. Hypoxia 2. Dehydration 3. Infection 4. Cold temperatures | |
| How does sickle cell disease present? | 1. AA pt, sudden severe pain in the chest, back, and thighs with fever. 2. Bilirubin gallstones 3. Recurrent infections from encapsulated organisms 4. Osteomyelitis 5. Retinopathy 6. Stroke | 7.Enlarged heart with hyperdynamic features and systolic murmurs. 8. Lower extremity skin ulcers 9. avascular necrosis of the femoral head. 10. Papillary necrosis of the kidney from chronic kidney damage. |
| What is the best initial test for sickle cell disease? | 1. Peripheral smear | |
| What is the most accurate test for sickle cell disease? | Hb electrophoresis | |
| What is the treatment for Sickle cell disease? | 1. Begin with oxygen/hydration/analgesia 2. IF fever or WBC count is high- then give ABX- Ceftriaxone, levofloxacin or moxifloxacin 3. Folic acid replacement is necessary on a chronic basis. 4. Give pneumococcal vaccine because of autosplenectomy | 5. Hydroxyurea prevents recurrences of sickle cell crisis by increasing HbF |
| How does a severe vasoocclusive crisis present? What is the treatment? | Presents with 1. Acute chest syndrome 2. Priapism 3. Stroke 4. Visual disturbance from retinal infarction Treatment: Exchange transfusion | |
| How does a pt with sickle cell trait present? | 1. Defect in the ability to concentrate urine - isothenuria 2.Can have hematuria 3. Clinically asx, normal CBC and normal smear 4. No treatment | |
| What is hereditary spherocytosis? | 1. There is a defect in the cytoskeleton of the red cell leading to an abnormal round shape and loss of flexibility, so RBC can't bend in the spleen | |
| What is the presentation of hereditary spherocytosis? | 1. Recurrent episodes of hemolysis 2. Intermittent jaundice 3. Splenomegaly 4. Family hx of anemia or hemolysis 5. Bilirubin gallstones | |
| What are the diagnostic test findings for hereditary spherocytosis? | 1. Low MCV 2. Increased MCHC 3. Negative Coombs test | |
| What is the treatment for hereditary spherocytosis? | 1. Chronic folic acid replacement supports RBC production 2. Splenectomy stops the hemolysis and does not eliminate the spherocytes. | |
| What diseases can cause Autoimmune (Warm or IgG) hemolysis? | 1. 50% are idiopathic 2. CLL/lymphoma 3. SLE | |
| What is the most accurate test for autoimmune hemolysis? | 1. Most accurate diagnostic test is the coombs test. | |
| Autoimmune hemolysis blood smear will show fragmented RBCs. T/F. | False. Smear does not show fragmented blood cells because destruction occurs in the spleen or liver.Not in the blood vessel. | |
| What is the treatment for autoimmune hemolysis? | 1. Glucocorticoids- prednisone, best initial therapy 2. Recurrent episodes respond to splenectomy 3. Severe, acute hemolysis not responding to steroids, tx with IVIG 4. Rituximab when splenectomy does not control hemolysis. | |
| What are the alternate agents used to diminish the need for corticosteroids? | 1. Cyclophosphamide 2. Cyclosporine 3. Azathioprine 4. Mycophenolate mofetil | |
| What is G6PD deficiency? | 1. X-linked recessive disorder leading to inability to generate glutathione reductase and protect the cells from oxidant stress. 2. Most common oxidant stress is infection | |
| How does G6PD present? | 1. AA pt or Mediterranean with sudden anemia and jaundice. 2. Normal sized spleen with an infection, or using meds that cause G6PD 3. | |
| What is the best initial test for G6PD? | 1. Best initial test in Heinz bodies and bite cells. | |
| What is the most accurate test for G6PD? | 1. G6PD levels will be normal after a hemolytic event. 2. G6PD levels 1 to 2 months after acute episode of hemolysis. | |
| What is the treatment for G6PD? | 1. Nothing reverses the hemolysis. 2. Avoid oxidant stress | |
| What is HUS and TTP? | 1.HUS and TTP are different versions of the same disease. 2. HUS associated with E.coli 0157:H7 and more frequent in children 3. TTP is associated with ticlopidine, clopidogrel, cyclosporine, and AIDS. | |
| What is the presentation of HUS? | 1. Intravascular hemolysis with fragmented red cells (schistocytes) 2. Thrombocytopenia 3. Renal insuffiency | |
| What is the presentation of TTP? | 1. Intravascular hemolysis with fragmented red cells (schisotocytes) 2.Thrombocytopenia 3. Renal insufficiency 4. Neuro disorder 5. Fever. | |
| What is paroxysmal nocturnal hemoglobinuria? | PNH- clonal stem defect with increased sensitivity of red cells to complement in acidosis. 2. Deficiency of CD 55 and 59 (DAF) 3. Defective gene is phosphatidylinositol class A | 4.During sleep, relative hypoventilation takes place, leading to mild increase in pCO2 an acidosis. 5. Normal person this has no effect but in PNH leads to hemolysis and thrombosis. |
| How does PNH present? | 1. Episodic dark urine with the first urination of the day from Hb. 2. Pancytopenia and iron deficiency anemia | |
| What is the most common cause of death in PNH? | Thrombosis | |
| What is the most accurate test for PNH? | 1. Decreased levels of CD 55 and CD 59, flow cytometry can be done. | |
| What is the treatment for PNH? | 1. Prednisone is the best initial therapy 2. Allogenic bone marrow transplant is the only cure 3. Eculizumab inactivates C5 in the complement pathway and decreases RBC destruction. | 4. Give supportive care such as folic acid replacement and transfusions as needed. |
| What is aplastic anemia? | 1. Aplastic anemia is pancytopenia of unclear etiology. 2. Any infection or cancer can invade the bone marrow causing decreased production or hypoplasia. | |
| What are causes of aplastic anemia? | 1. Radiation and toxins such as toluene, insecticides (DDT) and benzene 2. Drug effect: sulfa, phenytoin, carbamazepine, chloramphenicol, alcohol, chemo 3. SLE 4. PNH 5. Infection: HIV, hepatitis, CMV, EBV 6. B12 and folate deficiency | 7.Thyroid-inhibiting medications- PTU, methimazole |
| How to patients with aplastic anemia present? | 1. Fatigue due to anemia 2. Infections 3. Bleeding | |
| What is the most accurate test? | Bone marrow biopsy. Aplastic anemia is diagnosed after all other causes of pancytopenia are excluded | |
| What is the treatment of aplastic anemia? | 1. Supportive therapy-blood transfusions for anemia, 2. ABX for infections. 3. Platelets for bleeding 4. Treat underlying cause. 5. Allogenic BMT in a young patient is curative. | 6. If pt is above 50 or there is no donor, tx is antithymocyte globulin and cyclosporine 8. Tacrolimus is an alternative to cyclosporine. |
| PNH can lead to what disorders? | 1. Aplastic anemia 2. myelodysplasia 3. acute leukemia. | |
| What are causes of sideroblastic anemia? | 1.MCC is alcohol's suppressive effect on the bone marrow. 2. Lead poisoning 3. INH 4. Vit B6 deficiency | |
| What drugs can cause autoimmune hemolytic anemia? | 1. penicillin 2. alpha-methyldopa 3. rifampin 4. phenytoin. | |
| What are other causes of G6PD? | 1. Infections 2. Fava beans 3. Drugs- Dapsone, quinidine, sulfa drugs, primaquine, nitrofurantoin |
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