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Hematology

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