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Hematology
FA complete review part 3 Pathology
| Question | Answer |
|---|---|
| Another name for Acanthocytes? | "Spur cells" |
| Associated conditions with acanthocytes? | - Liver disease - Abetalipoproteinemia |
| What does 'acantho' stands for? | Spiny |
| What conditions exhibit Basophilic stippling? | Sideroblastic anemia and Thalassemia |
| Basophilic stippling is primarily seen in ___________________ (lab technique). | Peripheral smear |
| Which organ or area are ringed sideroblast most likely seen at? | Bone marrow |
| What are examples of Sideroblastic anemia causes? | Lead poisoning and Myelodysplastic syndromes |
| Technical name of "teardrop cells"? | Dacrocytes |
| What conditions are associated with teardrop RBCs? | Bone marrow infiltration (myelofibrosis), and thalassemias |
| Degmacytes = | "bite cells" |
| What is a more common name for Degmacytes? | "bite cells" |
| What pathology is seen with "bite cells"? | G6PD deficiency |
| Common name of Echinocytes | "burr cells" |
| Conditions seen with Burr cells? | - End-stage renal disease - Liver disease - Pyruvate kinase deficiency |
| How is an echinocyte different from an Acanthocyte? | Echinocytes' projections are more uniform and smaller. |
| What kind of RBCs are characteristic of G6PD deficiency? | "bite cells" |
| What is the type of RBC found in Pyruvate kinase deficiency? | Echinocytes or "burr cells" |
| Elliptocytes are seen with: | Hereditary elliptocytosis |
| Mutation in genes encoding RBC membrane proteins (spectrin) + elliptocytes. Dx? | Hereditary elliptocytosis |
| What are the characteristic RBCs in Megaloblastic anemia? | Macro-ovalocytes |
| In megaloblastic anemia, what are characteristic cells found? | Macro-ovalocytes (RBCs) and hypersegmented neutrophils |
| What condition is seen with Ringed sideroblasts? | Sideroblastic anemia |
| Excess iron in mitochondria is seen with what featured RBCs? | Ringed sideroblasts |
| Conditions with Schistocytes: | 1. Microangiopathic hemolytic anemias, including DIC, 2. TTP/HUS 3. HELLP syndrome 4. Mechanical hemolysis |
| What is an example fragmented RBCs? | Helmet cells |
| What precipitates sickling of RBCs? | Dehydration, deoxygenation, and at high altitude |
| Condition associated with Sickle cells | Sickle cell anemia |
| Description of Spherocytes | Small, spherical cells without central pallor |
| What conditions are seen with Spherocytes? | Hereditary spherocytosis, drug-and infection-induced hemolytic anemia. |
| Associated pathologies with Target cells | HbC disease, Asplenia, Liver disease, Thalassemia |
| Heinz bodies are seen in what pathology? | G6PD deficiency |
| What are the Heinz bodies? | Oxidation of Hb SH groups to -S--S to Hb precipitation |
| Hb precipitations seen in G6PD deficiency | Heinz bodies |
| What type of RBCs and RBC abnormalities are seen with G6PD deficiency? | Bite cells and Heinz bodies |
| What common RBC abnormality is seen in functional hyposplenia and or asplenia? | Howell-Jolly bodies |
| What are Howell-Jolly bodies? | Basophilic nuclear remnants found in RBCs |
| In a healthy individual, what cells remove Howell-Jolly bodies? | Splenic macrophages |
| What are the 3 main categories of anemias? | Microcytic, Normocytic, and Macrocytic |
| MCV < 80 fL. | Microcytic anemia |
| What MCV value accounts for microcytic anemias? | < 80 fL |
| What is the MCV range for normocytic anemias? | 80--100 fL |
| MCV 90 fL. Type of anemia? | Normocytic anemia |
| MCV value for Macrocytic anemias? | > 100 fL |
| What is the main defect for microcytic anemias? | Hemoglobin defects |
| Hemoglobin defects lead to development of ________________ anemias. | Microcytic anemia |
| What are the two types of Hemoglobin defects leading to Microcytic anemias? | 1. Defective heme synthesis 2. Defective globin chain |
| What are conditions/pathologies due to defective heme synthesis leading to microcytic anemias? | - Late iron deficiency - Lead poisoning - Sideroblastic anemia - Anemia of chronic disease |
| Thalassemias are due to _____________________ chain. | Defective globin chain |
| Which type of anemia are Thalassemias? | Microcytic anemia |
| Late iron deficiency lead to development of _____________ anemia. | Microcytic anemia |
| ____________ poisoning lead to microcytic anemia due to defective heme synthesis. | Lead |
| What metal toxicity lead to microcytic anemia due to defective heme synthesis? | Lead |
| Sideroblastic anemia is a __________________ anemia. | Microcytic anemia |
| List of Microcytic anemias: | 1. Late iron deficiency 2. Lead poisoning 3. Sideroblastic anemia 4. Anemia of chronic disease 5. Thalassemias |
| What are the main two divisions of Normocytic anemias? | 1. Non-hemolytic anemia 2. Hemolytic anemia |
| What measurement distinguish between Non-hemolytic and Hemolytic anemia? | Reticulocyte count |
| A Reticulocyte count of less or equal to 2%. | Non-hemolytic anemia |
| Types of Non-hemolytic, normocytic anemias: | 1. Early iron deficiency 2. Anemia of chronic disease 3. Aplastic anemia 4. Chronic kidney disease |
| Aplastic anemia is an example of an: | Non-hemolytic anemia |
| A patient with a long standing Hx of CKD, may develop what type of anemia? | Non-hemolytic anemia |
| The early stages of iron deficiency produces what kind of anemia? | Non-hemolytic anemia |
| What does the reticulocyte count must be in Hemolytic anemias? | > 2% |
| Hemolytic anemias are further divided into _____________ and _____________ anemias. | Intrinsic and Extrinsic |
| List of Extrinsic Hemolytic anemias: | 1. Autoimmune 2. Microangiopathic 3. Macroangiopathic 4. Infections |
| Microangiopathic and Microangiopathic produce _____________ | Extrinsic Hemolytic anemia |
| Autoimmune anemia and infection are causes of what kind of anemias? | Extrinsic Hemolytic anemia |
| What are the 3 main causes for Intrinsic hemolytic anemias? | 1. Membrane defects 2. Enzyme deficiencies 3. Hemoglobinopathies |
| Defects to the RBC's membrane will lead to development of ____________ _____________ anemia. | Intrinsic Hemolytic anemia |
| What 2 membrane defect conditions are associated with the development of intrinsic hemolytic anemias? | Hereditary spherocytosis and, Paroxysmal nocturnal hemoglobinuria |
| What kind of anemia is produced by Spherocytosis? | Intrinsic Hemolytic anemia |
| PNH produces _______ __________ anemia. | Intrinsic Hemolytic anemia. |
| What are two common diseases that lead to Intrinsic hemolytic anemias due to enzyme deficiencies? | 1. G6PD deficiency 2. Pyruvate kinase deficiency |
| Common hemoglobinopathies that cause Intrinsic hemolytic anemias? | Sickle cell disease and HbC disease |
| What are the two main types of macrocytic anemias? | Megaloblastic and Non-megaloblastic |
| Megaloblastic anemias are due to ______________ defects. | Nuclear |
| List of Non-megaloblastic anemias: | 1. Diamond-Blackfan anemia 2. Liver disease 3. Alcoholism |
| What type of anemia is an alcoholic is in risk of developing? | Non-megaloblastic anemia |
| What type of anemia is expected in a person with cirrhosis or liver disease? | Non-megaloblastic anemia. |
| Nuclear defects that produce megaloblastic anemias are of two primary kinds: | 1. Defective DNA synthesis 2. Defective DNA repair |
| Defective DNA ________ and ___________ lead to nuclear defects that produce megaloblastic anemias. | Synthesis and Repair |
| What conditions lead to a defective DNA synthesis, which is represented by megaloblastic anemia? | Folate deficiency Vitamin B12 deficiency Orotic aciduria |
| Vitamin B9 and B12 deficiencies produce: | Megaloblastic anemias |
| What type of anemia is Fanconi anemia? | Megaloblastic anemia |
| Defective DNA repair causing Megaloblastic anemia example: | Fanconi anemia |
| A patient with Orotic aciduria would develop what kind of anemia? | Megaloblastic anemia due to defective DNA synthesis |
| What are some causes of decreasing levels of iron? | - Chronic bleeding (GI loss, menorrhagia) - Malnutrition - Absorption disorders - GI surgery (gastrectomy) |
| What condition lead to Iron deficiency due to high demand? | Pregnancy |
| What are clinical presentations of Iron deficiency? | Glossitis, cheilosis, Plummer-Vinson syndrome |
| What is the triad of Plummer-Vinson syndrome? | Iron deficiency anemia Esophageal webs Dysphagia |
| What are common symptoms of Iron deficiency? | Fatigue, conjunctival pallor, pica, spoon nails |
| What is pica? | Consumption of nonfood substances |
| What is koilonychia? | Spoon nails |
| Pica is a common symptom of what condition? | Iron deficiency |
| What labs are increased in Iron deficiency? | TIBC, free erythrocyte protoporphyrin, and RDW |
| What labs are decreased in iron deficiency? | Iron and Ferritin |
| Ferritin levels in iron deficiency anemia are _________________. | Decreased |
| How are the TIBC levels in iron deficiency anemia? | Increased |
| alpha-globin gene deletions lead to a decrease in alpha globin synthesis. Dx? | a-Thalassemia |
| Cis deletion in alpha-thalassemia is seen in ____________ population mostly. | Asian |
| What population has more cases of alpha-thalassemia with a trans deletion? | African |
| How many alpha-globins are in HbH disease? | 1 alpha |
| (- -, -a). Dx? | Hemoglobin H disease |
| Hb Barts number of alpha-globin genes deleted: | 4 |
| 1 alpha-globin gene deleted. Dx? | alpha-thalassemia minima |
| 2 alpha-globin gene deleted. Dx? | alpha-thalassemia minor |
| What is the clinical outcome of Hb Barts? | Hydrops fetalis; incompatible with life |
| No a-globin, excess gamma-globin forms g-4? | Hemoglobin Barts disease |
| Excess B-globin forms Beta-4. Dx? | Hemoglobin H disease |
| What is the clinical outcome of HbH ? | Moderate to severe microcytic hypochromic anemia |
| If alpha-globin deletion/mutation occur in the same chromosome | Cis deletion |
| What are the types or kinds of B-thalassemias? | 1. B-thalassemia minor 2. B-thalassemia major 3. HbS/ B-thalassemia heterozygote |
| B-globin chain is underproduced + asymptomatic + Dx made by elevate HbA2. Dx? | B-thalassemia minor |
| Which B-thalassemia is homozygote? | B-thalassemia major |
| B-globin chain is absent. Dx? | B-thalassemia major |
| What are manifestations of B-thalassemia major? | Microcytic, hypochromic anemia with target cells and increased anisopoikilocytosis requiring blood transfusions |
| Why are B-thalassemia major patients often seen with Hemochromatosis? | Due to recurrent blood transfusions |
| What is the risk of developing with recurrent blood transfusions? | Secondary hemochromatosis |
| What condition (thalassemia) is featured with Marrow expansion ("crew-cut") on skull X-ray? | B-thalassemia major |
| "Chipmunk facies" Dx? | B-thalassemia major |
| What causes hepatosplenomegaly in B-thalassemia major? | Extramedullary hematopoiesis |
| What microcytic anemia condition is often seen with risk of developing Parvovirus B19-induced aplastic crisis? | B-thalassemia major |
| Why is B-thalassemia noticeable only after 6 months of been born? | HbF acts a protection |
| What type of hemoglobin is known to be protective of B-thalassemia major? | HbF |
| Mild to moderate sickle cell disease depend on amount of B-globin production. | HbS (B-thalassemia heterozygote) |
| What enzymes are inhibited in Lead poisoning? | Ferrochelatase and ALA dehydratase |
| What is the result of lead poisoning after ALA dehydratase and ferrochelatase are inhibited? | Decrease heme synthesis and increase RBC protoporphyrin |
| What causes the basophilic stippling in lead poisoning? | Due to inhibition of rRNA degradation leading to RBCs retaining aggregates of rRNA |
| What are the symptoms of LEAD poisoning? | 1. Lead lines on gingiva (Burton lines) and on metaphysis of long bones on x-ray 2. Encephalopathy and Erythrocyte basophilic stippling 3. Abdominal colic pain and sideroblastic anemia 4. Drops - wrist and foot drop |
| What are the 1st line of treatment for lead poisoning? | EDTA and Dimercaprol |
| What is used as chelating agent in kids for treatment of lead poisoning? | Succimer |
| What are the most common risks for Lead poisoning? | Exposure to old houses with chipped paint |
| Burton lines. Dx? | Lead poisoning |
| What type of bones are affected with lead lines in Lead poisoning? | Long bones metaphysis |
| What anemia is often seen along with Lead poisoning? | Sideroblastic anemia |
| What kind of "drops" are seen as common symptoms in Lead poisoning? | Wrist and Foot drops |
| Inhibition of ferrochelatase and ALA dehydratase. Dx? | Lead poisoning |
| X-linked defect in ALA synthase gene. Dx? | Sideroblastic anemia |
| What are the 3 types of causes for Sideroblastic anemia? | 1. Genetic (X-linked ALA synthase gene mutation) 2. Acquired (Myelodysplastic syndromes) 3. Reversible (MC- alcohol; lead, vit B6 deficiency, copper deficiency, isoniazid, and chloramphenicol) |
| What deficiencies are often associated with reversible sideroblastic anemia? | Vitamin B6 and Copper deficiencies |
| What is the MCC of reversible sideroblastic anemia? | Alcohol |
| What is the key histological feature of Sideroblastic anemia? | Ringed sideroblasts in bone marrow |
| What is the recommended treatment for Sideroblastic anemia? | Pyridoxine (B6, cofactor for ALA synthase) |
| Cofactor for ALA synthase | Pyridoxine |
| Levels of iron and ferritin are _____________ in Sideroblastic anemia. | Elevated |
| Findings of Megaloblastic anemias: | RBC macrocytosis Hypersegmented neutrophils Glossitis |
| Description of megaloblastic anemia | Impaired DNA synthesis leads to: maturation of nucleus of precursor cells in bone marrow delayed relative to maturation of cytoplasm |
| What are most common causes of Folate deficiency? | - Malnutrition (alcoholics) - Malabsorption - Drugs (MTX, TMP, Phenytoin) - Increased requirement (hemolytic anemia, pregnancy) |
| What are the most significant labs of Folate deficiency? | - Elevated homocysteine |
| Which amino acid level is elevated in Folate deficiency? | Homocysteine |
| Which megaloblastic anemia causes are seen with NO neurologic symptoms and normal methylmalonic acid levels? | Folate deficiency |
| How is the level of methylmalonic acid in Folate deficiency megaloblastic anemia? | Normal |
| What is another name for Vitamin B12? | Cobalamin |
| What are the MCC of Vitamin B12 deficiency? | 1. Pernicious anemia 2. Malabsorption (Crohn disease) 3. Gastrectomy 4. Insufficient intake (veganism) 5. Diphyllobothrium latum (fish tapeworm) |
| Which surgical procedure increase risk of Vitamin B 12 deficiency? | Gastrectomy |
| Vegans for a extended period of time are at higher risk of developing what anemia? | Megaloblastic anemia due to Vitamin B12 deficiency |
| Fish tapeworm causative of Megaloblastic anemia due to cobalamin deficiency? | Diphyllobothrium latum |
| Elevated homocysteine and Elevated methylmalonic acid. Dx? | Megaloblastic anemia due to Vitamin B12 deficiency |
| Which vitamin B deficiency has elevated levels of methylmalonic acid? | Vitamin B12 |
| Does Folate or Cobalamin deficiency present megaloblastic anemia with neurological symptoms? | Cobalamin |
| What are the associated neurologic symptoms of Vitamin B12 deficiency causing megaloblastic anemia? | - Reversible dementia - Subacute combined degeneration |
| What condition gives rise to Subacute Combined Degeneration? | Vitamin B12 deficiency |
| Why does Cobalamin deficiency cause Subacute combined degeneration? | B12 involvement in fatty pathway and myelin synthesis |
| What tracts are affected in Subacute combined degeneration? | 1. Spinocerebellar tract 2. Lateral corticospinal tract 3. Dorsal column dysfunction |
| At what stage of the Schilling test is Vitamin B12 deficiency diagnosed? | Stage 4 |
| Diagnosed cobalamin deficiency in stage 4 is due to: | Dietary insufficiency |
| Which organ may harvest vitamin B12 reserve for several years? | Liver |
| What is depleted faster, Folate or Cobalamin? | Folate |
| Inability to convert orotic acid to UMP because of defect in UMP synthase. Dx? | Orotic aciduria |
| What pathway is affected in Orotic aciduria? | de novo pyrimidine synthesis pathway |
| AR disorder; Presents with FTT, developmental delay, and megaloblastic anemia refractory to folate and B12; No hyperammonemia. Dx? | Orotic aciduria |
| No hyperammonemia + Megaloblastic anemia. Dx? | Orotic aciduria |
| Elevated levels of Orotic acid and Hyperammonemia. Dx? | Ornithine transcarbamylase deficiency |
| If only orotic acid is elevated and normal levels of ammonia. Dx? | Orotic aciduria |
| What is the MC treatment for Orotic aciduria? | Uridine monophosphate or uridine triacetate to bypass mutated enzyme |
| RBC macrocytosis without hypersegmented neutrophils. | Non-megaloblastic anemia |
| What are common features of Diamond-Blackfan anemia? | Short stature, craniofacial abnormalities, an upper extremity malformations (triphalangeal thumbs). |
| What condition is seen with elevated HbF but overall decrease in Hb? | Diamond-Blackfan anemia |
| What type of anemia is seen with macrocytosis but DNA synthesis is normal (unimpaired)? | Non-megaloblastic anemia |
| Hemolysis can lead to increases in: | LDH, reticulocytes, unconjugated bilirubin, urobilinogen in urine. |
| What are the main findings of Intravascular hemolysis? | Decreased haptoglobin and increased schistocytes on blood smear. |
| What the most characteristics symptoms of intravascular hemolysis? | Hemoglobinuria, hemosiderinuria, adn urobilinogen in urine. |
| What kind of of hyperbilirubinemia is seen in intravascular hemolysis? | Unconjugated hyperbilirubinemia |
| What are findings Extravascular hemolysis? | Macrophages in spleen clear RBCs |
| The increase in hepcidin in anemia of chronic disease leads to: | Decrease release of iron from macrophages and decreased iron absorption from gut. |
| What is the function of hepcidin? | Released by liver, binds ferroportin on intestinal mucosal cells and macrophages, thus inhibiting iron transport |
| What is the main cause of Aplastic anemia? | Failure or destruction of myeloid stem cells |
| What are some causes of myeloid stem cell destruction that cause Aplastic anemia? | 1. Radiation and drugs 2. Viral agents 3. Fanconi anemia 4. Idiopathic |
| What are associated drugs that lead to Aplastic anemia? | Benzene, chloramphenicol, alkylating agents, and antimetabolites. |
| What are viral agents associated as a cause of Aplastic anemia? | EBV, HIV, hepatitis viruses |
| What is the cause of Fanconi anemia? | DNA repair defect causing bone marrow failure; macrocytosis |
| What are some characteristics features of Fanconi anemia? | 1. Short stature 2. Increase incidence of tumors/leukemia 3. Cafe-au-lait spots 4. Thumb/radial defects |
| What labs seen with Aplastic anemia? | Decreased reticulocyte count, and increased EPO |
| What is Pancytopenia? | Anemia, leukopenia, and thrombocytopenia. |
| Histology of Aplastic anemia? | Normal cell morphology, but hypocellular bone marrow with fatty infiltration |
| Dry bone marrow tap + "weird" shaped thumbs. Dx? | Aplastic anemia due to Fanconi anemia |
| What is Hereditary spherocytosis? | Extravascular hemolysis due deficit in proteins interacting with RBC membrane skeleton and plasma membrane. |
| Premature removal of RBCs from spleen due to membrane skeleton structure mutations. Dx? | Hereditary spherocytosis |
| What are the characteristic labs seen in Hereditary spherocytosis? | - Increased fragility in osmotic fragility test - Normal to decreased MCV with abundance of cells |
| What are findings seen with Spherocytosis? | Splenomegaly and aplastic crisis (Parvovirus B19 infection) |
| Most common enzymatic disorder of RBCs? | G6PD deficiency |
| What type(s) of hemolysis are seen in G6PD deficiency? | Extravascular and Intravascular hemolysis |
| Inheritance mode of G6PD deficiency? | X-linked recessive |
| Which pathology is seen with reduced glutathione due to enzymatic defect? | G6PD deficiency |
| Hemolytic anemia following oxidant stress. DX? | G6PD deficiency |
| Patient presents with back pain, hemoglobinuria a few days after starting malarial treatment. Dx? | G6PD deficiency |
| What are the histological remarks found in G6PD deficiency? | Bite cells with Heinz bodies |
| Drugs and other substances that precipitate G6PD deficiency | Sulfa drugs, antimalarial, infections, and fava beans |
| What condition is seen with RBC increased susceptibility to oxidant stress? | G6PD deficiency |
| Description of RBCs in Pyruvate kinase deficiency | Rigid RBCs |
| AR disorder; rigid RBCs and extravascular hemolysis. MVC of 86 fL. Dx? | Pyruvate kinase deficiency |
| Hemolytic anemia in a newborn | Pyruvate kinase deficiency |
| What are the effects on 2,3-BPG and oxygen affinity in Pyruvate kinase deficiency? | Increased levels of 2,3-BPG lead to lower Hb affinity for oxygen |
| Acquired mutation in PIGA gene. Dx? | Paroxysmal nocturnal hemoglobinuria |
| Pathogenesis of PNH? | Increased complement -mediated intravascular RBC lysis |
| Impaired synthesis of GPI anchor for decay-accelerating factor and membrane inhibitor of reactive lysis that protects RBC membrane from complement | Pathogenesis of Paroxysmal Nocturnal Hemoglobinuria |
| Triad in Paroxysmal nocturnal hemoglobinuria? | 1. Coombs (-) hemolytic anemia 2. Pancytopenia 3. Venous thrombosis |
| Treatment for Paroxysmal nocturnal hemoglobinuria? | Eculizumab |
| What condition is treated with Eculizumab? | Paroxysmal nocturnal hemoglobinuria |
| (+) for CD55/59 (-) RBCs on flow cytometry | Paroxysmal nocturnal hemoglobinuria |
| How does Eculizumab work? | Inhibits terminal complement formation |
| Defective GPI factor synthesis. Dx? | Paroxysmal nocturnal hemoglobinuria |
| What condition is associated with mutated or defective decay-accelerating factor? | Paroxysmal nocturnal hemoglobinuria |
| What amino acid replaces glutamic acid in Sickle cell anemia? | Valine |
| Which chain in Sickle cell anemia is the one with the amino acid replacement? | B-globin chain |
| Hemolysis present in Sickle cell anemia | Extravascular and Intravascular hemolysis |
| What is the pathogenesis of Sickle cell anemia? | Low O2, high altitude, or acidosis precipitates sickling leading to anemia, vaso-occlusive disease. |
| Why are Sickle cell anemia newborns are asymptomatic? | Increased HbF and decreased HbS |
| What is an important characteristics of heterozygotes of Sickle cell anemia? | Resistance of malaria |
| Another way to refer to heterozygous sickle cell anemia. | Sickle cell trait |
| Crescent-shaped RBCs | Sickle cells |
| What causes the "crew cut" on x-ray of sickle cell disease? | Marrow expansion form increased erythropoiesis |
| What are the painful crises of sickle cell disease? | Dactylitis, priapism, acute chest syndrome, avascular necrosis, stroke. |
| What is dactylitis? | Painful swelling of hands/feet |
| What is the result of Sickling in renal medulla in sickle cell patients? | Renal papillary necrosis leading to microhematuria. |
| What is the most common osteomyelitis cause in Sickle cell anemia? | Salmonella osteomyelitis |
| What is the treatment of Sickle cell disease? | Hydroxyurea and Hydration |
| What is the purpose of treating SCD with hydroxyurea? | Increase HbF |
| What condition is treated by inducing increase of fetal hemoglobin with hydroxyurea? | Sickle cell disease |
| HbC disease is due to: | Replacement of Glutamic acid with Lysine. |
| What amino acid replaces glutamic acid in HbC disease? | Lysine |
| What type of hemolysis is seen in HbC disease? | Extravascular hemolysis |
| Which is milder form of disease, HbSC or HbSS? | HbSC |
| What is the difference between HbSC and HbSS? | HbSC has only one mutant gene |
| What are the findings of HbC disease homozygotes blood smear? | Hemoglobin Crystals inside RBCs, and Target cells |
| Hemoglobin crystals inside RBCs. Dx? | HbC disease |
| Which are the two types of autoimmune hemolytic anemia? | Warm and Cold anemia |
| Chronic anemia seen in SLE and CLL and with certain drugs. | Warm (IgG) autoimmune hemolytic anemia |
| Acute anemia triggered by cold. | Cold (IgM and complement) autoimmune hemolytic anemia |
| Common drug that may trigger Warm autoimmune hemolytic anemia? | a-Methyldopa |
| Associated conditions with Warm hemolytic anemia? | SLE and CLL |
| What are some associated malignancies and/or infection that lead to Cold hemolytic anemia? | - CLL - Mycoplasma pneumoniae infections - Infectious mononucleosis |
| Which extrinsic hemolytic anemia is seen with RBC agglutination that cause painful, blue fingers and toes with cold? | Cold (IgM and complement) autoimmune hemolytic anemia |
| Autoimmune hemolytic anemias are usually ________________. | Coombs (+) |
| What is the Direct Coombs test? | Anti-Ig antibody (Coombs reaganet) added to patient's RBCs |
| What happens in Direct Coombs test if patient's RBCs are coated with Ig? | RBCs agglutinate |
| Normal RBCs added to patient's serum. | Indirect Coombs test |
| When do RBCs agglutinate in Indirect Coombs test? | If serum (patient) has anti-RBC surface Ig, RBCs agglutinate when Coombs reagent is added. |
| If antibody is added to patient's cells, Direct or Indirect Coombs test? | Direct Coombs test |
| If normal RBCs are added to patient's serum, Direct or Indirect Coombs test? | Indirect Coombs test |
| AIHA stands for? | AutoImmune Hemolytic Anemia |
| What is the patient's component in Direct Coombs test? | RBCs, =/- anti-RBC Ab |
| Patient component in Indirect Coombs test | Patient serum ; +/- anti-donor RBC Ab |
| What is the reagent in Direct Coombs test? | Anti-human globulin |
| Anti-human globulin = | Coombs reagent |
| (+) Result in Direct Coombs test means: | Agglutination Anti-RBC Ab is PRESENT |
| What would be the result of a Direct Coombs test, if the patient has no anti-RBC Ab? | Negative (no agglutination) |
| What are the reagents of Indirect Coombs? | 1. Donor blood 2. Coombs reagent |
| Agglutination in Indirect and Direct Coombs, always means? | Positive (+) result |
| What needs to be present in Indirect Coombs test to yield a positive result (agglutination)? | Anti-donor RBC Ab |
| What is the pathogenesis of Microangiopathic anemia? | RBCs are damaged when passing through obstructed or narrowed vessel lumina. |
| What conditions are associated with Microangiopathic anemia? | DIC, TTP/HUS, SLE, HELLP syndrome, and hypertensive emergency. |
| What featured RBC are often present in Peripheral Blood smear in Micro-/Macroangiopathic anemias? | Schistocytes |
| Prosthetic heart valves and aortic stenosis may cause hemolytic anemia secondary to mechanical destruction of RBCs. | Pathogenesis of Macroangiopathic anemia |
| Microangiopathic and Macroangiopathic anemias are ________ ____________ anemias. | Extrinsic Hemolytic |
| What are two common infections that cause extrinsic Hemolytic anemia? | Malaria and Babesiosis |
| What is the primary disturbance in Iron deficiency? | Decreased serum iron |
| Primary disturbance in anemia of chronic disease | Increased Ferritin |
| What is the main or primary disturbance in Hemochromatosis? | Increased serum iron |
| What is the primary disturbance in a pregnant woman or one using OCPs? | Increased levels of Transferrin or TIBC |
| What is the role of TIBC? | Indirectly measures transferrin |
| Primary iron storage protein of body. | Ferritin |
| What is the role of Transferrin? | Transports iron in the blood |
| What protein transports iron in blood? | Transferrin |
| What are the most common Leukopenias? | Neutropenia, Lymphopenia, and Eosinopenia |
| Absolute neutrophil count < 1,500 cells/mm3 | Neutropenia |
| At what neutrophil count do severe infections most commonly occur? | < 500 cells/mm3 |
| What are some common causes of Neutropenia? | Sepsis/post-infection, drugs, aplastic anemia, SLE, and radiation |
| A person in chemotherapy is often found to develop which leukopenia? | Neutropenia |
| Lymphopenia cell count ---> | Absolute lymphocyte count < 1,500 cells/mm3 |
| Common causes of lymphopenia: | HIV, DiGeorge syndrome, SCID, SLE, corticosteroids, radiation, sepsis. |
| How low does the Eosinophil absolute count need to go to be considered eosinopenia? | < 30 cells/mm3 |
| What syndrome is associated with Eosinopenia? | Cushing syndrome |
| What effect do corticosteroids have in neutrophil count? | Neutrophilia |
| Corticosteroids cause _________________, __________________, and __________________. | Eosinopenia, Lymphopenia, and Neutrophilia |
| What is a "Left shift"? | Shift to a more immature cell in the maturation process. |
| What is Leukoerythroblastic reaction? | Left shift seen with immature RBCs |
| Left shift of neutrophils gives rise to what precursors? | Band cells and metamyelocytes |
| How do corticosteroids cause Eosinopenia? | Sequester eosinophils in lymph nodes and cause the apoptosis of lymphocytes |
| How do corticosteroids cause Neutrophilia? | They impair the activation of neutrophil adhesion molecules, impairing the migration out of the vasculature to sites of inflammation. |
| Term given to the increase neutrophil precursors, such as band cells and metamyelocytes. | Left shift |
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rakomi
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