UWorld 2017 Flashcards
Complication of Chronic Granulomatous Disease
Recurrent suppurative infections with Catalase-positive organisms
2 consequences of C1 deficiency
Increased susceptibility to infection with encapsulated bacteria
Predisposes to SLE
C3 deficiency predisposes to
Recurrent infections with encapsulated bacteria
C5-9 deficiency leads to
Infections with N. meningitidis and N. gonorrhoeae
Defect in type I interferon release leads to
Increased susceptibility to viral infections
5 causes of pancytopenia without splenomegaly
Aplastic anemia Vit B12 deficiency anemia Folic acid deficiency anemia Acute leukemias Certain forms of myelodysplastic syndrome
Primary thrombotic microangiopathy syndromes
Platelet activation + diffuse microthrombosis in arterioles/capillaries
Hemolytic anemia w/ schistocytes
Thrombocytopenia
Organ injury (brain, kidney, heart)
Pentad of Thrombocytopenic thrombotic purpura
Fever Neuro symptoms (progr lethargy) Renal failure Anemia Thrombocytopenia In the setting of GI illness + normal TP, aPTT, BT
Vinca alkaloids
Act on M phase of cell cycle
Neurotoxicity (peripheral neuropathy)
Sickle cell anemia
Point mutation: glutamic acid instead of valine (in beta-chain of Hb)
Exertional dyspnea
Pneumonia w/ life-threatening acute chest syndrome
Recurrent abdo pain
Heparin-induced thrombocytopenia
HIT treated w/ direct thrombin inhibitors (argatroban)
Avoid HMWH + LMWH
Chronic myeloproliferative disorders
Mutation in JAK2 (cytoplasmic tyrosine kinase)
Persistent activation of signal transducers + activators of transcription (STAT) proteins
Side effects of Dapsone (prophylactic ttt of HIV+ for pneumocystic jiroveci pneumonia in case of TMP-SMX allergy)
Fever, rash, methemoglobinemia, oxidative stress
If G6PD deficiency, hemolytic anemia
Immunophenotyping of lymphoblasts in precursors B-ALL and T-ALL
B-ALL: TdT+, CD10+, CD19+
T-ALL: TdT+, CD1a+, CD2, CD3, CD4, CD5, CD7, CD8
Intravascular hemolytic anemia
Decreased serum haptoglobin
Increased LDH
Increased indirect bilirubin
Cause of Polycythemia vera
Majority have JAK2 V617F mutation
Hematopoietic stem cells more sensitive to growth factors
Labo in HUS
Decreased hemoglobin, platelets
Increased bleeding time, lactate dehydrogenase, bilirubin, BUN, creatinine
Activating mutation of KRAS gene
Constitutive activation of EGFR
Increased cell prolif + growth
Resistant to chemotherapy w/ anti-EGFR drugs (cetuximab, panitumumab)
Labo in lead poisoning
Microcytic anemia w/ normal iron studies
Basophilic stippling
Serious complication of chronic hemolytic anemia + frequent blood transfusion
Hemosiderosis
Hemosiderin (brown-yellowish pigments) in Kupffer cells in liver
After various anticancer agents, tumor cells can become resistant
Human multidrug resistance (MDR1) gene
P-glycoprotein: transmembrane ATP-dep efflux pump protein
P-GP: reduce influx of drugs into cytosol, increase efflux from cytosol
So prevent action of CT
Growth factors for angiogenesis in neoplastic + granulation tissue
VEGF and FGF
Proinfl cytokines (IL1, IFN-G) indirectly by increased VEGF
Laminin in basement membranes: barrier to new Vx formation
Chronic vs acute leukemias on peripheral blood smears
Chronic: prevalence of mature cells
Acute: prevalence of blasts
Protein C deficiency
Exaggerate hypercoagulability after Warfarin: short 1/2 life of prot C + def of prot C
Thrombotic occlusion of microvasculature w/ skin necrosis
Hairy cell leukemia
Indolent B-cell neoplasm
Bone marrow failure
Infiltration in reticuloendoth system: massive splenomegaly
Dry tap of BM + lymphocytes w/ cytoplasmic projections
Splenic changes in sickle cell ds
Repeated splenic infarctions
Then splenic atrophy + fibrosis (late ado): autosplenectomy
Then inf w/ encaps bact
Megaloblastic anemia
Severe macrocytosis (MCV > 110) Folic acid or vit B12 def Chronic hemolytic anemia: increased erythrocyte turnover so increased folic acid requirements; risk of macrocytosis
Aplastic anemia
Triad: low Hb, thrombocytopenia, absent hematopoietic cells in BM
Increased circulating EPO if normal renal fct
Pure red cell aplasia
Rare form of marrow failure
Severe hypoplasia of marrow erythroid elements
Normal granulopoiesis + thrombopoiesis
Ass w/ thymoma, lymphocytic leukemias, parvo B19 inf
DIC
Complication of G- bact sepsis, acute pancreatitis, burn injury, APL
G- bact endotoxins activate coag cascade
Schistocytes, thrombocytopenia
Decreased fibrinogen, prolonged PT + PTT
AML, M3 presentation
Can present w/ persistent inf + coagulopathy: hgic signs
BM biopsy: promyelocytes w/ Auer rods
t(15;17) translocation: fusion of retinoic acid receptor-alpha gene + promyelocyte leukemia gene
Follicular lymphoma
Overexpression of bcl-2
Increased secretion of Bcl-2 prot: inhibits apoptosis
Infections in sickle cell ds
Strepto pneumoniae
Hemophilus influenza
Osteomyelitis: #1 Salmonella, #2 Staph aureus and E coli
Sickle cell trait
Asymptomatic
Relative protection from malaria
Normal Hb, reticulocyte, RBC index values
Life expectancy = gen pop
B-cell vs T-cell ALL
B-cell: 70-80%, fever, malaise, bleeding, bone pain, HSM
T-cell: 15-17%, mediastinal mass, resp sympt, dysphagia, SVC sd
Ttt of hereditary spherocytosis
Splenectomy + blood transfusions
Folic acid supplementation
Hemophilia
Low fact VIII or IX: failure to convert prothrombin to thrombin
Adding thrombin to blood: clotting
