Science Quiz / BCM heme onc

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Can you name the BCM heme onc?

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name the disorder: hypersegmented polys, ineffective erythropoiesis, increased MCV, buildup of homocysteine & methylmalonyl-CoAB12 deficiency. may also see spacticity & reduced proprioception. folate deficiency rare b/c of dietary fortification (also would not cause buildup of methylmalonyl-CoA)
first-line chemo cocktail for hodgkinsadriamycin, bleomycin, vinblastine, dacarbazine. may use MOPP in older patients. also, usually add XRT. excellent survival rates w/ treatment
name the disorder: spherocytes on smear, acquired condition; DAT+; IAT - (usually); IgG+, C3 +/-tx: steroids (work in 60%), splenectomy (works in 2/3 of adults), immunosuppression. can be seen in CLL/SLL.
name the heritable (x-linked) anemia: hemolysis in response to flava beans, primaquine, sulfamethoxazole, moth balls, oxidizing agentsheinz bodies in RBCs w/ crystal violet stain, blister and bite cells
name the plasma protein: upregulated in anemia of chronic disease; degrades ferroporininduced by cytokines. traps iron in gut epithelium & macrophages. high ferritin, low transferrin & TIBC. microcytic, hypochromatic.
preferred treatment for hemophilia Aplasma-derived factor VIII is used for vWF deficiency (has some vWF), but this lower-risk treatment is preferred for hemophilia A.
MDS most commonly transforms into which other neoplasmcalled 'AML with multilineage dysplasia'
name the disorder: Inherited (AR) anemia due to DNA-repair disorder; cafe au lait spots, microophthalmia, abnl thumbs, aplastic anemiapredisposed to AML/MDS, other cancers
chemotherapy cocktail for aggressive NHLrituximab, cyclophosphamide, Hydroxydaunorubicin, oncovin, prednisone. can also add radiotherapy.
name the heritable (AD) anemia 2-2 defect in ankyrin. extravascular hemolysisbits of membrane torn off by RES. indirect hyperbilirubinemia, reticulocytosis, splenomegaly, negative coomb's test, osmotic fragility of RBCs
name the drug: direct GPIIb/IIIa inhibitorticlopidine & clopidogrel inhibit GPIIb/IIIa indirectly but reducing expression in platelets
name the disorder: congenital mutation of hepcidin or HFE gene; reduced hepcidin activityHIGH iron. 5-7:1 male:female, presents in 4th dcade. cardiomyopathy, arthritis. to manage, phlebotomize regularly & avoid dietary iron.
t(14;18) leading to bcl-2 expression found 85% of cases of which intermediate grade NHL?still chronic, but more aggressive than CLL/SLL. occurs in older folks. often just treat symptoms--tough to cure.
Neutrophils' primary granules contain myeloperoxidase, lysozyme, elastase. What do secondary granules contain? eosinophil granules contain peroxidase, & major basic protein. basophils' contain histamine & heparin.
this coagulation assay measures the function of factors VII-->X-->V-->II>Ithis is the extrinsic + common pathway. used for monitoring warfarin therapy using the INR.
name the disorder: acquired HA, can be triggered by TTP, HUS, DIC, disseminated carcinomatosis, obstetric complications, vasculitis, Kasabach-Merrit, Shear stress on artifical valvon peripheral smear, will see schistocytes & helmet cells
name the rare disorder of episodic intravascular hemolysis 2-2 acquired mutation in PIG-A gene; RBCs lose membrane bridges & defense against complementCD55 & CD59 missing from RBC membrane. Causes Budd-Chiari, weird thrombi. treat w/ corticosteroids or BMT
name the disorder: congenital RBC aplasia; present in 1st yr of life w/ macrocytic, hypoproductive anemia; paucity of erythroid precursors in otherwise normocellular marrow85% respond to steroids; reason why is unclear. eosinophilia common. may have other congenital abnl'ities
Hb A2 is less than 3.5% in nl adults. Elevated in which condition?A2 is alpha2delta2. moderately elevated in trait; WAY elevated in B-thal major.
this coagulation assay is prolonged in hemophilia A & B (& C!!)XII-->XI-->IX-->VIII-->X-->V-->II-->I. intrinsic & common pathways. monitor heparin therapy.
this chromosomal abnormality is seen in 95% of CML, 5% of pediatric ALL and 25% of adult ALLtranlocation (9;22). imatinib (gleevec) has revolutionized CML treatment; presence of chromosome still worsens prognosis in ALL.
this factor does not cause bleeding but does prolong PT/PTT. identified w/ DRVVT and hexagonal phase phospholipid neutralization test.misnomer: not an anticoagulant in vivo, and not always associated w/ lupus.
Hb SS and SB0 give a severe sickle cell phenotype. Aside from AS (sickle cell trait), which 2 combinations (most commonly) give a mild phenotype?B0 and B+ are the total and partial knockout classes associated with beta thalassemia (though both classes contain many indivual mutations). C results from a single point mutation common in African Americans; it can participate in sickling..
name the AML class: inversion (16). good prognosis, 'trash mouth'gum infiltration, sometimes DIC. good prognosis.
Name the factor: paucity leads to normochromic normocytic anemia deficiency seen in renal insufficiency. prevents apoptosis of erythroid precursors.
the L&H or 'popcorn' cell is the malignant cell of variant hodgkin's disease; what b-cell derivative is the malignant cell of classical hodgkins?RS cells are rare; many more reactive lymphocytes, eos, & others
Cis-platinum causes nephrotoxicity. Which chemo drug causes hemorrhagic cystitis?vinca alkaloids, taxanes, and platinum compounds can cause peripheral neuropathy
Right or left shift of Hb saturation curve: high temp, high 2,3-BPG, low pH, low affinity hbHb Kansas, exercise
Favorable mutation in CLL: deletion of short arm of this chromosomedel(11q), del(17p), or t(11q;various) worsen prognosis. Trisomy 12 or the lack of detectable mutation are neutral prognostically.
name the drug for sickle cell disease increased production of Hb F, which inhibits sicklingreduces frequency of painful crises
hintanswerextra fun facts
Fragmentation hemolysis, thrombocytopenia, fluctuating neurological changes, renal insufficiency, and fever comprise the classic pentad of clinical findings in what condition?now the diagnostic triad includes fragmentation hemolyis, thrombocytopenia, and elevated LDH. best treatment: plasma exchange, corticosteroids. good: immunosuppression, vincristine, splenectomy. bad: platelet transfusion, anti-platelet drugs, DDAVO
Name the AML class: translocation (15;17). unique treatment with all-trans-retinoic acid.acute promyelocytic leukemia. lots of auer rods. big risk of DIC. otherwise good prognosis for AML. only subclass that gets consolidation chemo.
name the rounded, oval, or rod-shaped pale grayish-blue inclusions in neutrophil cytoplasm, associated w/ pregnancy, infections, neoplasms, burns, trauma, G-CSF therapy, Kwashiorkostacks of RER or denatured aggregates of free ribosomes
Name the AML class: translocation (8;21), most common subclassAML with maturation. good prognosis for AML.
Adriamycin causes cardiotoxicity. Which chemo drug causes pulmonary fibrosis?these are both in ABVD for Hodgkins
Bernard-Soulier is a congenital defect of platelet receptor GP Ib which causes bleeding & macrothrombocytopenia. Glanzmann thrombasthenia is a congenital defect of which platelet rthis is the fibrinogen receptor. Glanzmann shows defective aggregation to all agonists except ristocetin; Bernart-Soulier shows decreased platelet aggregation with ristocetin.
name the disorder: agglutinated RBCs on peripheral smear, ischemia of fingers & earlobes, anemiaDAT- in half; need cold agglutinin titer! IgG - , C3+
name the precipitations of denatured Hb found in RBCs in alpha thalassemia and G6PD deficieny
name the disorder: pruritis when taking a hot bathmutation in JAK2; cells grow in absense of epo
name the disorder: dual population of RBCs, pseudo pelger-huet WBCs, high serum iron & %sat & ferritin.iron not incorporated into porphyrin. acquired or congenital. ringed sideroblasts (iron in mitochondria). tx w/ pyridoxine & transfusions.
vWF deficiency is a common inherited bleeding disorder. type 2 is a qualitative defect; types 1 & 3 are quantitative. which is more severe, type 1 or type 3?this is the autosomal recessive form (with 2N). treatment w/ DDAVP, synthetic vasopressin, is more effective in type I.
Patient w/ sickle cell disease has MCA flow rate lower than 170 cm/s. Good sign or bad sign?High flow velocity means there's a smaller diameter due to partial obstruction. Fast flow = stroke risk. Transfusions lower velocity & stroke risk.
name the pathology: soccer ball cells in PBSmost common blood cancer, older folks, anemia & thrombocytopenia; also likely to see smudge cells
name the disorder: blue people, single aa mutation in Hb shifts the plane of heme & causes oxidation of FeNOT methemoglobinopathy! that is 2-2 a mutation of reducing enzymes, not Hb itself
name the neoplasm of plasma cells presenting with hypercalcemia, renal insufficiency, anemia, & lytic bone lesionsoften see rouleaux (a sign of elevated protein) on peripheral smear
name the syndrome defined by bone marrow hypocellularity (25%) and 2 of: neutrophils below 500/uL; anemia w/ retics less than 40k/uL; platelets less than 20k/uLTx: HSCT if pt is less than 40 yo and has matched sib donor. Otherwise, immunosuppression. MUD earlier in kids or adults who fail ATG twice
common cause of teardrop cells (along with myelophthisic anemia)myelophthisis is infiltration of bone marrow by outside (usually malignant) tissue
Pulmonary HTN is major mortality risk in sickle cell disease. Do medications indicated for primary pulmonary HTN reduce mortality?other causes of mortality in sickle cell: stroke, chest syndrome, renal failure, hepatic failure, sudden death during painful crises
neoplasm associated with herpes zoster, fungal infections, and fluctuating 'Pel Ebstein' fever B symptoms common. t-cell immune deficiency can continue after lymphoma is treated.
name the anemia: low Hb, HCT, MCV, retic count; hypochromatic RBCs; pencil cells; poikylocytosisalso low serum iron, ferritin, transferrin sat; high serum transferrin & TIBC. can have thrombocytosis or thrombocytopenia
name the basophilic nuclear remnants found in rbcs in asplenia or functional hyposplenia
specific growth factor promoting granulocyte development during myelopoiesiserythropoietin: RBCs, thrombopoietin: platelets
name the anticoagulant drug w/ side effects of bleeding, osteopenia, and induced thrombocytopeniadrug binds to platelet factor-4; IgG forms against new antigen
name the disorder: hemoglobin H (and/or Bart's) presentHb H is a tetramer of beta chains, toxic to RBC membrane. precipitates in micro heinz bodies to make 'golf ball cell.' only seen with 3 gene copy deletions (4=fetal death).
fastest growing human cancer, associated w/ EBV, c-myc linked to one of the Ig genes(8;14); (2; 8); (8;22). 'starry sky' histology. adolescents in Africa.
first site of fetal hematopoiesis: 16-20 daysaortogonadonephros 20-30 days; liver & placent 6 wks; thymus migration 7-8 wks; bone marrow 5 mos
Where's the hemolysis? High LDH & indirect bilirubin, low haptoglobinalso urine Hb & hemosiderin--taken up by renal tubular cells which are shed into urine
name the anticoagulant that can cause embryopathy (6-12 wks gestational age) and skin necrosismonitor w/ PT & INR. Antidotes = plasma & vitamin K
RBC cytoplasmic inclusion commonly seen in thalassemias, anemia of chronic disease, iron deficiency, and lead poisoningaggregations of RER and ribosomes
Name the channel: overactive in young RBCs, dehydrates the cells, increases Hb S concentration & rate of sicklingRBCs in sickle cell disese are younger (more Gardos activity). Gardos pumps out K+, and water follows. Sickling is concentration dependent.

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