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USMLE2 Medicine 09


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 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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