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USMLE2 Medicine 09
Hematology
| Question | Answer |
|---|---|
| Definition of anemia in men, in women (Hct and Hb) | men: Hct<41 or Hb<13.5; women Hct<36 or Hb<12 |
| EtOH-induced anemia - microcytic, normocytic or macrocytic? | Macro - huge RBC's to hold all the liquor! |
| Fe-def anemia - microcytic, normocytic or macrocytic? | Micro - take out the Fe and you have smaller RBCs! |
| Vitamin B12 deficiency anemia - microcytic, normocytic or macrocytic? | Macro |
| thalassemia - microcytic, normocytic or macrocytic? | Micro |
| anemia of chronic disease - microcytic, normocytic or macrocytic? | either micro OR normocytic |
| Pb poisoning - microcytic, normocytic or macrocytic anemia? | microcytic |
| Folic acid deficiency - microcytic, normocytic or macrocytic? | macrocytic |
| Sideroblastic anemia - microcytic, normocytic or macrocytic? | microcytic |
| liver disease - microcytic, normocytic or macrocytic anemia? | macrocytic |
| methotrexate - microcytic, normocytic or macrocytic anemia? | macrocytic |
| AZT - microcytic, normocytic or macrocytic anemia? | macrocytic |
| hemolysis - microcytic, normocytic or macrocytic anemia? | normocytic |
| Microcytic anemia (4) | I'm feeling very small (micro) -- I need TLC. Iron def, Thalassemia, Lead, Chronic disease |
| normocytic anemia, increased retic (4) | It's normal to be SICK of the 6th HEMi SPHERe: Hemolysis, hereditary Spherocytosis, G6PD, Sickle cell |
| normocytic anemia, decreased or normal retic (3) | early Fe def or chronic disease, also Primary Bone Marrow disorder |
| Macrocytic anemia (7) | MR BIDHL is pale (anemia) and fat (macro). Myelodysplastic syndrome, Reticulocytosis, B12 def, Iron def, Drugs (EtOH), Hypothyroid, Liver disease |
| basophilic stipling | thalassemia or lead --> anemia |
| schistocytes | MAHA |
| bite cells | G6PD (removal of the Heinza bodies from RBC's) |
| Heinz body prep | G6PD (Heinz bodies - DNA inclusions in RBC's) |
| Schilling test | to diagnose B12 deficiency |
| Drugs that cause anemia | EAT HiM 5x to give him anemia. EtOH, AZT, TMP/SMX, Hydroxyurea, Methotrexate, 5-FU |
| positive osmotic fragility test | Hereditary spherocytosis |
| Positive red cell enzyme essay | pyruvate kinase def or G6PD |
| positive direct coombs | immune hemolytic anemia |
| HAM test (acidified - hemolysis test) | paroxysmal nocturnal Hburia |
| anti-globulin testing | paroxsymal cold Hburia |
| positive urine hemosiderin means.... | intravascular hemolysis |
| negative urine hemosiderin means... | extravascular hemolysis - in macrophages, liver, spleen, etc |
| Hct should rise how many points per one unit of RBC's | 3 |
| Most common reason for Fe def anemia | blood loss (GI or menstrual) |
| type of murmur in Fe def | systolic flow murmur |
| lab dx of Fe def anemia (ferritin, TIBC, MCV, serum Fe, retic, RDW) | low ferritin, high TIBC (liver making more to max available Fe usage), low MCV, low serum Fe, low retic, high RDW |
| Tx for Fe def anemia | oral Fe; parenteral for malabsorbers or those who need lots of Fe |
| lab dx of anemia of chronic disease (ferritin, TIBC, MCV, serum Fe, retic) | nl or hi ferritin (Fe sequestered in stores in ret-endoth system), MCV micro or normocytic, TIBC low, serum Fe low, retic low |
| Tx for anemia of chronic disease | correct the underlying disease |
| T/F: anemia of chronic disease treated with Fe | F. Fe won't do any good since it's not Fe they're lacking |
| T/F: anemia of chronic disease treated with EPO | F. Fe won't do any good since it's not APO they're lacking, unless part of the chronic disease is renal dysfunction |
| Anemia: faulty Hb synthesis b/c Fe trapped in mitochondria of nucleated RBC's | sideroblastic anemia |
| lab dx of sideroblastic anemia (ferritin, TIBC, MCV, serum Fe) | high ferritin (Fe trapped in mitochondria), low TIBC (very high transferrin saturation, so low binding capacity), low serum Fe, low MCV (microcytic) |
| defect in aminolevulinic acid synthase causes.... | hereditary sideroblastic anemia |
| abnl vit B6 metabolism causes.... | hereditary sideroblastic anemia |
| Drugs that can cause sideroblastic anemia | CIA: chrloramphenicol, INH, alcohol |
| Myelodysplastic syndrome associated with what malignancy? | AML |
| Most specific test for sideroblastic anemia | Prussian Blue stain of RBC's - find ringed sideroblasts |
| Tx for hereditary sideroblastic anemia | pyridoxine, give iron chelation for Fe overload from Fr trapped in mitochondria |
| underproduction of globin chains | thalassemia |
| alpha thalassemia most common in what population? | Asians |
| beta thalassemia most common in what population? | Mediterranean |
| Hb A | a2 B2 |
| HbF | a2 g2 |
| HbA2 | a2 D2 |
| HbH | B4 |
| Bart's Hb (what are the globin and genetic profiles?) | g4 2/2 3-gene deletion of alpha chains |
| 6 month old baby that becomes severely anemic | B thal major (Cooley) anemia (6 months is when body switches from fetal Hb to adult Hb - starts to use B chains) |
| child with growth failure, HSMegaly, jaundice, body deformities, and anemia | B thal major (Cooley) anemia. Bony deformities due to extramedullary hematopoiesis |
| hair on end sign | extramedullary hematopoiesis. SITS: sickle cell, thalassemia major, iron deficiency anemia, and spherocytosis |
| pt that is dependent on qweekly/qmonthly transfusions | B thal major |
| lab dx of thalassemia (Fe, ferritin, TIBC, RDW, MCV, PBS) | nl Fe, ferritin, TIBC, RDW. low MCV. PBS - target cells |
| which thalassemia with increased HbF and HbA2? | beta |
| which thalassemia with normal HbF and HbA2? | alpha |
| Tx for B thal major | qweekly/qmonthly transfusions, deferasirox (oral Fe chelation for Fe overload 2/2 repeated transfusions), splenectomy to eliminate major area of hemolysis, some can get bone marrow transplant |
| tapeworm that can cause anemia | Diphyllobothrium latum --> decreased absorption --> B12 deficiency --> macrocytic anemia |
| anemia and neurologic deficits | vitamin B12 deficiency |
| anemia with hypersegmented WBC's | B12 or folic acid deficiency |
| macrocytic anemia with oval RBC's | B12 or folic acid deficiency |
| Can B12 def anemia be treated with folic acid? | Folic acid can treat the hematologic abnormalities, but not the neurologic sx's |
| Lab diagnosis of B12/folic acid def anemia (Fe, retic, bone marrow, cell count, other - 3) | Fe inc, retic dec, hypercellular bone marrow, pancytopenia, inc LDH, inc bili, inc methylmalonic acid) |
| EtOH causes what kind of anemia and why? | Macrocytic because dec folic acid intake |
| what anticonvulsant drug can cause macrocytic anemia? | phenytoin |
| chronic hemolytic anemia | SPH: sickle cell, PNHburia, hereditary spherocytosis |
| acute hemolytic anemia | DAG: drug-induced, autoimmune, G6PD deficiency |
| Haptoglobin. What is its role and what is the lab test used for? | HPT binds free Hb. HPT-Hb removed by spleen. Intravascular hemolysis → free Hb in blood → increased HPT-Hb binding → low HPT. Extravascular hemolysis (spleen) → released Hb phagocytosed → HPT not used up → no HPT levels. |
| substitution of valine for glutamic acid | Hb S - sickle cell (substitution in B globin chain) |
| sickle cell crisis precipitated by what? | hypoxia, dehydration, acidosis, infection, fever |
| sudden increase in hemolysis | thing G6PD or splenic sequestration |
| What causes sudden drop in Hct | Parvovirus B19 or folic acid deficiency (acute aplasia - not hemolysis) |
| how to tx sickle cell pain crisis + fever | CTX and cefotaxime (covers Strep PNA and HFlu), IVF, pain control, O2 |
| how to tx sickle cell acute chest syndrome/priaprism/CNS dysfxn/cardiac | RBC transfusion if Hct is low, exchange transfusion if Hct is hi |
| Chronic management of sickle cell pt | folic acid replacement, vaccinations against Strep PNA and HFlu, hydroxyurea, bone marrow transplant - still experimental |
| cold agglutinin disease | IgM against RBC --> increased hemolysis in liver (this is why steroids and splenectomy can't treat) |
| what diseases is cold agglutinin disease associated with? | cold Wet MULE: Waldenstrom macroglobulinemia, mycoplasma, UC, lymphoma, EBV |
| pt with cyanosis of ears, nose, fingers, and toes | cold agglutinin disease |
| pt with weakness, pallor, jaundice, dark urine | think hemolysis |
| use Coombs test in which types of anemia? | autoimmune, cold agglutinin, drug-induced hemolysis |
| spherocytes on blood smear | autoimmune hemolysis |
| lab dx of autoimmune hemolysis (MCV, retic, LDH, haptoglobin, bili) | nl MCV and retic, inc LDH and unconj bili, dec haptoglobin |
| Tx for autoimmune hemolysis | steroids, then splenectomy if refractory |
| Tx for cold agglutinin disease | avoid cold --> drugs (CAC - azathioprine, cyclosporine, cyclophosphamide). hemolysis in liver (this is why steroids and splenectomy can't treat) |
| loss of spectrin in cell membrane | hereditary spherocytosis |
| genetics of hereditary spherocytosis | autosomal dominant |
| lab dx of Hereditary Spherocytosis (MCV, MCHC, LDH, bili, retic, Coombs) | low MCV, hi MCHC, hi LDH and bili, hi retic, NEG Coombs |
| Osmotic fragility test | Hereditary spherocytosis (inc sensitivity to lysis in hypotonic sol'ns |
| Tx of hereditary spherocytosis | daily folate --> removal of spleen if severe |
| Decay accelerating factor (aka CD55 and CD59) | low in paroxysmal nocturnal Hburia |
| pt with dark urine in the AM and venous thrombosis | paroxysmal nocturnal Hburia because overnight --> hypoventilation --> acidosis --> intravascular hemolysis |
| RBC membrane defect in phosphatidyl inositol glycan A (PIG-A) | paroxysmal nocturnal Hburia |
| paroxysmal nocturnal Hburia | faulty PIG-A --> RBC membrane defect --> inc binding of complement to RBC --> inc intravascular hemolysis with acidosis |
| paroxysmal nocturnal Hburia can lead to what? | aplastic anemia and leukemia |
| Budd Chiari syndrome | occlusion of the hepatic veins, presents with the classical triad of abdominal pain, ascites and hepatomegaly |
| sugar water test | paroxysmal nocturnal Hburia - shows increased susceptibility to lysis by complement |
| tx of paroxysmal nocturnal Hburia | Fe replacement for urinary blood loss, steroids, anticoag if thromboses |
| drugs causing oxidative stress --> G6PD | P-NSDQ: Sulfa, primiquine, dapsone, quinidine, nitrofurantoin |
| lab dx G6PD def (LDH, Bili, MCV, retic, haptoglobin) | inc LDH bili and retic. nl MCV, dec hapt |
| tx G6PD | hydration and avoiding oxidant stress |
| anemia, leukopenia, thrombocytopenia | aplastic anemia, pancytopenia |
| marrow of someone with aplastic anemia | absence of precursor cells, hypoplastic and fat filled, no abnormal cells |
| what is classically associated with aplastic anemia? | CRaB eats up the cells in your marrow! benzene, radiation, chloramphenicol |
| What is the most common cause of aplastic anemia? | idiopathic |
| Tx of aplastic anemia | if <50, then allogeneic (stem cell) bone marrow transplant (80-90% cure). if no BMT, then immunosuppression (CAP: cyclosporine, antithymocyte globulin, prednisone) |
| most common leukemia in children | ALL |
| most common leukemia in children | AML |
| DIC associated with which leukemia? | M3 promyelocytic leukemia |
| CNS involvement is associated with which leukemias (2) | M4 and M5 monocytic leukemia |
| leucostasis | WBC extremely elevated --> sludging of WBC's in vasculature --> HA, SOB, confusion, brain hemorrhage |
| dx of acute leukemia | bx showing >20% blasts |
| auer rods | AML (M3) |
| myeloperoxidase and esterase associated with which leukemia? | AML |
| CALLA antigen | ALL |
| terminal deoxynucleotidyl transferase (TdT) | associated with ALL |
| Tx regimen for acute leukemia | 1. aim for remission (remove 99.9% leukemic cells from body and elim of peripheral blasts in circulation), 2. consolidation, 3. BMT if relapse |
| chemo for AML | Arabinoside + Danorubicin |
| chemo for ALL | DVP: daunorubicin, vincristine, prednisone |
| Tx for promyelocytic leukemia | DVP: daunorubicin, vincristine, prednisone PLUS vitamin A deriv (all trans retinoic acid) |
| Tx for leukostasis | leukapheresis and chemo |
| prophylaxis of CNS involvement in ALL | intrathecal methotrexate |
| what do you find in CML? | overproduction of myeloid cells (neut, eos, baso), elevated WBC's with pred neutrophils and left shift |
| Philadelphia chromosome | CML |
| what is the genetic defect in CML | philadelphia chromosome - translocation between chromosomes 9 and 22 --> enzyme with tyrosine kinase activity |
| most common sx's of CML | fatigue, night sweats, fever, abdominal pain from spleen enlargement |
| leukocyte alkaline phosphatase score (LAP) low | CML. This is what distinguishes CML from leukemoid reaction |
| best initial therapy for CML, secondary therapy | imatinib (Gleevec) - inhibitor of tyrosine kinase. if no response, then BMT |
| asymptomatic elevation of WBC's (80-98% lymphocytes) on routine evaluation of a pt 50yo | CLL |
| Stage 0 CLL | lymphocytosis only (LLSAT) |
| Stage 1 CLL | LAD (LLSAT) |
| Stage 2 CLL | splenomegaly (LLSAT) |
| Stage 3 CLL | anemia (LLSAT) |
| Stage 4 CLL | thrombocytopenia (LLSAT) |
| CLL associated with.... | autoimmune hemolytic anemia |
| CD19 associated with.... | CLL |
| Smudge cells on PBS | CLL |
| Why is staging important in CLL? | Stages 0-1: 10-12 years survival without treatment, Stages 3-4 1-2 year survival |
| Tx for CLL (early, symptomatic, advanced) | early stage with only lymphocytosis or LAD is not treated. Always tx if symptomatic. if advanced - fludarabine. |
| if CLL and autoimmune hemolysis and TCP | tx appropriately + prednisone. |
| CLL relapse | use pentostatin |
Created by:
christinapham
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