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HemeOnc Q's

QuestionAnswer
What does "Neutrophils Like Making Everything Better" stand for? WBC diff highest to lowest: neutrophils, lymphocytes, monocytes, eosinophils, basophils
What does the large SA:volume ratio in RBCs help facilitate? easy gas exchange
what is the main source of energy in RBCs 90% anearobically from glucose to lactate, 10% from HMP shunt
What causes the physiologic chloride shift and what does the chloride shift do? chloride bicarb antiport system,, allows transport of C02 from periph to lungs
anisocytosis varying sizes
poikilocytosis varying shapes
What is the life span of a normal RBC 120 days
From what cells are platelets derived from megakaryocytes
what do platelets interact with to form a hemostatic plug fibrinogen
What do the dense granules contain in platelets ADP and Ca
What the alpha granules contain in platelets? vWF and fibrinogen
Where are 1/3 of platelets stored spleen
What is the life spance of a platelet? 8-10 days
What is the result of thrombocytopenia or platelet dysfunction? petechiae
What is the receptor for vWF Gp1b
What is the receptor for fibrinogen? GpIIb/IIIa
what are the azuraphilic granules in PMNs lysosomes
What is contained within the azurophilic granules of PMNs hydrolytic enzymes, lysozyme, myeloperoxidase, lactoferrin
Where do you see hypersegmented polys? B12/folate def
What percentage if WBCs are polys? 40 to 75
What percentage of WBCs are monocytes? 2 to 10
Where are monocytes typically found, where do they go, and what do the differentiate into? blod, tissue, MACS
What signal activates MACS IFN gama
What percentage of WBCs are eosinophils? 1 to 6
What do eosinophils defend against and what do they use to do it? helminth infections major basic protein
Eosinophils are highly phagocytic for what kind of complex? antigen-antibody
what are the two substances produced by eosinophils help limit the reaction following mast cell degranulation? histaminase and arylsulfatase
What does NAACP stand for, in regards to the causes of eosinophiia? neoplastic, asthma, allergic processes, collagen vacsular disease, parasites
What percentage of WBCs are basophils, and what is found in their basophilic granules? < 1%, heparin, histamine, LTD-4, other vasoactive amines
What substances are released in mast cell degranulation histamine, heparin, and eosinohil chemotactic factors
What portion of IgE can mast cells bind Fc
Where are mast cells found tissue
Where are basophils found? blood
What substance prevents mast cells degranulation? cromolyn sodium
Who are the professional APCs? dendritic cells?
What are dendritic cells called in the skin? langerhans cells
What do they express on their surface? MHC II and Fc receptors
What is their role? inducers of primary antibody response
How are the nucleus and the cytoplasm characterized for lymphocytes round densly staining nucleus with a small amount of pale cytoplasm
Where do B cells arise from, mature, and migrate to marrow, marrow, follicles of lymph nodes, white pulp of spleen (unencapsulated lymphoid tissue)
Can B cells function as APCs? Yes, via MHC II
How are plasma cells characterized? Off center nuclues, clock face chromatin
Plasma cell neoplasm multiple myeloma
What do plasma cells do produce antibody, lots of RER and golgi
Where to T cells arise and mature, and migrate to bone marrow, thymus, blood (80% of circulating lymphos are T)
Blood type A A antigen and B antibodies
Blood type B B antigena and A antibodes
universal recipient AB, no antibodies
universal donor no antigen, both antibodies
In a Rh- mother who has developed anti Rh antibodies, who is at risk of hemolytic disease? her next Rh+ fetus
What is the treatment to prevent a woman from forming anti Rh antibody? Rhogam, Rh antigen immunoglobulin
Which maternal antibodies cross the placenta, anti A/B or anti Rh anti Rh
What begins the extrinsic pathway? tissue factor converst the VII and VIIa
What activates the intrinsic pathway? collagen, BM, activated platelets
What activates the fibrinolytic pathway? XIIa activates kallikrein, which activates plasmin (and converts HMWK to bradykinin
What does plasmin do? degrades fibrin mesh and converts C3 to C3a
What does bradykinin do? inc vasodiltion, inc perm, inc pain
What is hemophiliia A deficiency in factor VIII
What is hemophilia B? def in factor IX
What does Vit K deficiency cause? dec synthesis of factors 1972, protein C/S
What does antithrombin do and what activates it? inhibits thrombin, ixa, xa, xiia, and activated by heparin
What is the purpose of the fibrin mesh acts to stabilize platelet plug
What is the effect of ACE on bradykinin inactivates it
Which enzyme converts vit k to activated vit k, and what substance inhibits this enzyme epoxide reductase, warfarin inhibits
Which factor does vWF carry/protect VIII
What role does antithrombin play? incactivates II, VII, IX, X, XI, XII
What activates protein C and what does activated protein C do? protein S, cleaves and invactivates Va and VIIIa
What is factor V leidin? factor V resistant to activated protein C's inhibition
What converts plasminogen to plasm and what does plasmin do? tPA, cleavage of fibrin mesh
After an injury, what does vWF bind to begin platelet plug formation? exposed collagen upon endothelial damage
What do the platelets bind? What is the step called they bind vWF via GpIb
What do platelets release necessary for coagulation cascaed? ADP and Ca
What does ADP do? helps platelts adhere to endothelium
What is the activation stage of platelet plug formation? ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
What is the aggregation phase of ppf? fibrinogen bind GpIIb/IIIa and links platelts
What are the pro aggregation factors? TXA2, dec blood flow, inc platelet aggregation
what are the anti aggregation factors? PGI2, NO inc blood flow, dec platelet aggregation
Deficiency in GpIb bernard soulier
Deficiency in vWF von Willebrand's disease
Deficiency in GpIIb/IIIa Glanzmann's throbmasthenia
Drug that inhibits COX and therefore TXA2 synthesis aspirin
drug that inhibits ADP induced expression of GpIIb/IIIa ticlopidine/clopidogrel
Drug that inhbits the GpIIb/IIIa directly abciximab
Acanthocyte (spur cell) liver disease, abetalipoproteinemia, acntho
Basophilica stippling TAIL, thalassemias, anemia of chronic disease, iron def, lead poisoning
Bite cell G6PD
Elliptocyte hereditary elloptocytosis
Macro-ovalocyte megaloblastic anemia (also hypersegmented PMNs, marrow failure)
Ringed sideroblasts Sideroblastic anemia
Schisotcyte, helmet cell DIC, TTP/HUS, traumatic hemolysis
Sickle cell Sickle cell anemia
Spherocyte hereditary spherocytosis, autoimmune hemolysis
Teardrop cell Bone marrow infiltration, myelofibrosis
Target cell HbC disease, Asplenia, Liver disease, thalassemia, "HALT the hunter said to his target"
Oxidation of iron, denatured hemoglobin preceipitation damage to RBC membrane, formation of bite cells Heinz bodies, seen in alpha thal and G6PD
Basophilic nuclear remnants fonud in RBCs Howell-Jolly bodies, hypo/asplenia
Ddx for microcytic anemia fe def, thal, pb poison, sideroblastic anemia
iron deficiency anemia, esophageal web, atrophic glossitis plummer-vinson syndrome
Causes of iron def? bleeding, malnutrition/absorption, inc demand pregs
defect in alpha globin gene, dec alpha globin synth, disease and populations? alpha thal, asian and african american
What are the four levels of alpha thal? defect in 4, not compatible with life, defect in 3, HbH disease, defect in 1/2 not associated with sig anemia
What causes hydrops fetalis Hb Barts, gamma4 in defect in all 4 alpha genes
What is HbH beta 4
What is the defect in beta thal? splicing sites and promotor sequences
What happens in betal thal minor? beta chain underproduced, asymptomatic, heterozygote
How is beta thal minor dx inc HbA2 on electrophoresis
What happens in beta thal major? beta chain absent, homozgote, severe anemia requiring blood transfusion, secondary hemochromatosis
Marrow expansion, "crew cut" on skull x ray, skeletal deformaties, and chipmunk faces beta thal
inc HbF on electrophoresis both alpha and beta thal
inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis, also inhibition of rRNA degradation lead poisoning, rRNA causes aggregration of ribosomes and basophilic stippling
X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis sideroblastic anemia
What is the treatment for sideroblastic anemia B6 therapy (pyrodixine)
What reveresible things can a sideroblastic anemia lead, and EtOH
What do you see on peripheral smear with sideroblastic anemia, ringed sideronblasts with iron laden mitochondria
What do the iron studies show in sideroblastic anemia inc serum iron, normal TIBC, inc ferratin
What does LEAD stand for in lead poisoning? Lead lines on gingivae and epiphyses of long bones on xray, Encephalopathy and Erythrocyte basophilic stippling, Abdominal colic and Anemia, Drops -wrist and foot drop,
What is the treatment for lead poising? Dimercaprol and EDTA 1st line of treatment, succimer for kids - it sucks to be a kid who eats lead
What is the Ddx for for a macrocytic anemia Folate/B12, nonmegaoblastic anemias, liver dz, EtOH (non folate/B12) reticulocytosis, metabolic disorder, drugs
What is a metabolic disorder tht can cause macrocytic anemia? orotic aciduria
Which drugs can cause macrocytic anemia? 5-FU, AZT, hydroxyurea
What is the general pathology of a macrocytic anemia? DNA synth impaired, maturation of nucleus delayed relative to maturation of cytoplasm
hypersegmented PMNs, glossitis, dec folate, inc homocysteine, nl methylmalonic acid folate def
What are the etiologies of folate def malnutrition, malabsoprtion, impaired metabolism, inc requirement as in an hemolytic anemia or pregnancy
hypersegmented PMNs, glossitis, dec B12, inc homocysteine, inc methymalonic acid B12 def
What are the etiologies of B12 def insufficient intake as with strict vegans, malabsorption as in crohns, pernicious anemia, diphyllobothrium
Why does B12 def cause neuro sx? B12 in fatty acid pathways leads to subacute combined degeneration
What are the neuro sx of B12 def? Peripheral neuropathy, posterior columns (vibration/proprioception), lateral corticospinal (spasticity), dementia
What is the Ddx for a normocytic, normochromic anemia? Hemolytic or non hemolytic, and then for the hemolytiuc, intravascular hemolysis or extravascular hemolysis
dec haptoglobin, inc LDH, hemoglobin in urine (paroxysmal nocturnal hemoglobinuria, mechanical destruction as in aortic stenosis or a prosthetic valve intravascular hemolysis
inc LDH, jaundice, extravascular hemolysis
What causes the jaundice in extravascular hemolysis inc UCB
What are some classic examples of extravascular hemolysis hereditary spherocytosis, G6PD, sickle cell
What is the Ddx for nonhemolytic normocytic anemia Anemia of chronic disease, Aplastic anemia, kidney disease
What is the pathogenesis of ACD? inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe, dec TIBC, inc ferritin
hypocellular bone marrown tih fatty infiltration aplastic anemia, pancytopenia
What is the ddx for aplastic anemia radiation/drugs, viral agents, fanconi's anemia, idiopathic may follow acute hepatitis
fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection signs and sx of aplastic anemia
what is the tx for aplastic anemia allogenic bone marrow transplant, RBC/platelet transfusion, C-GSF, or GM-CSF
What is the pathogenesis of aplastic anemia with kidney disease decrease EPO
Defect in proteins interacting with RBC membrane skeleton and plasma membrane hereditary spherocytosis
Is HS extravascular or intravascular? extravascular
what proteins can be defective in HS? ankryin, band 4.1 or spectrin
What virus can cause an aplastic crisis in pts with HS? parvovirus
What finding you do you see in patients after splenectomy Howell Jolly bodies
What are the labs and tx for HS? positive osmootic fragility test and splenectomy
X linked, dec glutathione inc RBC susceptibility to oxidative stress G6PD
Fava beans, sufla drugs, infectinos, examples of oxidative stress
Back pain, hemoglobinuria common a few days after oxidative stress in a pt with G6PD
What do you see in peripheral smear in a pt with G6PD? bite cells and Heinz bodies
Is G6PD intravascular or extravascular intravascular
hemolytic in a newborn, dec ATP and rigid RBCs pyruvate kinase def, extravascular
Crew cut on skull xray due to marrow supression, 8% of AA population, single amino acid replacement in beta chain at position 6, after a DNA point mutation Sickle cell
Why can newborns with sickle cell be asymptomatic inc HbF and dec HbS
What is the pathogenesis of sickle cell deoxygenated HbS polymerizes, low O2 or dehydration precipiates sickling, anemia and veno-occlusive disease
Aplastic crisis after parvovirus, autosplenectomy, salmonella osteomyelitis, painful crisis, renal papillary necrosis, splenic sequestration complications of sickle cell anemia
What causes renal papillary necrosis in sickle cell low O2 in papilla; can also get microhematuria from medullary infarcts
What is the tx for sickle cell hydroxyurea, bone marrow transplant
What is the mutation in HbS valine for glutamate
What is the mutation in HbC lysine for glutamate at position 6
Who has more severe disease, HbSS or HbSC HbSS
Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho paroxysmal nocturnal hemoglobinuria
What are the extrinsic hemolytic normocytic anemias? ...
What are some causes of warm agglutinin autoimmune hemolytic anemia? SLE, CLL, alpha methyldopa
Which immunoglobulin is involved in warm agglutination? IgG, Warm is GREAT
What are some causes of cold agglutinin anemia Cold, seen in CLL, mycoplasma pneumoniae infections or infectious mononucleosis
mother's antibodies attack fetal RBCs erythroblastosis fetalis, Rh or other blood antigen incompatibility
What is the coombs test results in AIHA? pos
anti-Ig antibody added to patients RBCs; direct coombs, agglutinate if RBCs are coated with Ig
normal RBCs added to patient's serum indirect coombs, agglutinate if serum anti RBC surface Ig
RBCs damagaed from passing through obstructed, narrowed vessel lumina MAHA
When do you see MAHA? DIC, TTP-HUS, SLE malignant HTN, prostethic heart valves, aortic stenosis
What is the characteristic finding for MAHA on peripheral smear? schistocytes, helmet cells
Which infections can cause MAHA? malaria, Babesia
Serum iron, transferrin, ferritin lab values for iron def anemia down, up, down
Serum iron, transferrin, ferritin lab values for anemia of chronic disease down, down, up
Serum iron, transferrin, ferritin lab values for hemochromatosis up, down, up
Serum iron, transferrin, ferritin lab values for pregs, OCP use nl, up, nl
Serum iron, transferrin, ferritin lab values for lead poisoning anemia up, down, nl
Conditions of defective heme synthesis leading to accumulation of heme precurors porphyrias
What is the affected enzyme in lead poisoning ferrocheletase and ALA dehydrogenase
What is the accumulated substance in lead poisoning protoporphyrin (blood)
What is the are the presenting symptoms of lead poisoning in kids and adults Kidney, Gi, microcytic anemai in both, kids
What are the likely exposures of kids and adults for lead poisoning kids
What is the effected enzyme in acute intermittment porphyria? porphobilinogen deaminase aka uroporphyrinogen I synthase
What is the affected enzyme in acute intermittment porphyria porphobilinogen, delta ALA, uroporphyrin
What are the presenting symptoms of acute intermittent porphyria painful abdomen (also in lead poisoning), red wine colored urine, polyneuropathy, psychological distrubances, precipated by drugs,
What is the treatment for acute intermittent porphyria glucose and heme, which inhibit ALA synthase
What is the enzyme defect in Porphyria cutanea tarda uroporphyrinogen decarboxylase
What substance accumulates in porphyria cutanea uroporphyrin (tea colored urine)
What are the presenting symptoms of porphyria cutanea tarda blistering cutaneous photosens, most common porphyria
what does decreasing heme do to ALA synthase activity? inc
What does increasing heme do to ALA synthase activity dec
Which substrates begin the heme synthesis pathway glycine and succinyl-coa
What vitamin is a cofactor for the first step of heme synthesis B6
What substance is Fe added to to yield heme protoporphyrin
Which pathway and factorrs are tested by the PT coag test extrinsic, I, II, V, VII and X
Which pathway and factors are tested in the PTT test intrinsic, all factors except, VII, XIII
In hemophilia A or B what do you see in the coag tests nl PT, elevated PTT - intrinsic pathway defect
What do you see in vit K def eleveated PT, PTT
What is the clinical picture of hemophilia A or B macrohemorrhage, hemarthroses, easy bruising
How do platelet disorders present? inc bleeding time, microhemorrhage: mucuous membrane bleeding, epitaxis, petechiae, purpura, possible thrombocytopenia
Defect in platelet to collagen adhesion, receptor, disorder, platelet count and BT Gp1b, bernard-soulier, dec platelets (giant platelets not counted), inc BT
Defect in platelet to platelet adhesion, receptor, disorder, platelet count and bleeding time Glanzmann's throbmasthenia, GpIIb/IIIa, nl platelets, inc BT
What does the blood smear show in glanzmann's? no platelet clumping
anti-GpIIb/IIIa antibodies leading to peripheral platelet destruction Idiopathic thrombocytopenia (ITP)
What do labs show in ITP? dec platelet survival and inc megakaryocytes, thrombocytopenia and inc BT
deficiency in ADAMTS13 leading to dec degradation of vWF thrombotic thrombocytopenic purpura
What is the presenting scenario for TTP? PENTAD: neurologic sx, renal sx, fever, thrombocytopenia, MAHA
What is the pathogenesis of TTP? inc large vWF multimers, inc platelet aggregation and thrombosis
What do the labs show for TTP? schistocytes and inc LDH
Intrinsic pathway coagulation defect and defect in platelet plug formation, most common inherited bleeding disorder, AD vWD
How does vWD cause elevated PTT? vWF carries/protects factor VIII
What is the tx for vWD DDAVP (desmopressin) which releases stored vWF stored in endothelium
Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states DIC
What does STOP Making New Thrombi stand for Causes of DIC: Sepsis (gram neg), Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Tranfusion
What do labs show in DIC? schistocytes, inc fibrin split products (D-dimers), dec fibrinogen, dec factors V and VIII
Production of mutant factor V that cannot by degraded by protein C, most common cause of inherited hypercoaguability Factor V Leiden
Mutation in 3' untranslated region associated with venous clots prothrombin gene mutation
Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin ATIII def
dec ability to activate factors V and VIII, inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin Protein C or S deficiency
lymphoid neoplasms with widespread involvement of bone marrow, tumor cells usually foind in peripheral blood Leukemia
discrete tumor masses arising from lymph nodes lymphoma
inc WBC count with left shift, inc leukocyte alkaline phosphatase, often due to infection leukemoid reaction
What is the characteristic histologic finding in Hodgkin Lymphoma Reed-sternberg cells
What is the characteristic spread of Hodgkin Lymphoma contiguous
What symptoms are associated with Hodgkin lymphoma B symptoms - fever night sweats, weight loss
Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy Hodgkin
What virus is associated with 50% of Hodgkin lymphoma EBV
What is the age breakdown for hodgkins bimodal, men>women, except for nodular sclerosing type
What indicated a good prognosis in Hodgkin lymphoma? inc lymphoctes, and less RS cells
When is the peak incidence for nonHod lymphoma 20 to 40
What cell is primarily involved in non Hod lymph B cells
What is the pattern of involvement and spread for nonHod lympho multiple, peripheral nodes, extranodal involvement, noncontiguous spread
What state is commonly associated with nonHod lymphoma HIV or immunosupression
What are the variants of the RS cells lacunar in nodular sclerosing variant
What CD molecules are on RS cells CD15 and CD30 pos
What is the most common hodgkin lymphoma nodular sclerosing
t(8;14) c-myc gene Burkitt lymphoma
What virus is associated with Burkitt lymphoma EBV
What is the difference of presentation of Burkitt in Africa vs the United States Africa
What do you see a starry sky appearance in Burkitt sheets of lymphocytes interspersed with macs
What is the most common nonhodgkin lymphoma Diffuse Large b cell lymphoma
CD5+, poor prognosis, t(11;14) Mantle cell, older males
bcl-2 t(14;18) adults follicular lymphoma, indolent course
HTLV-1 adult T cell lymphoma, presents with cutaneous lesions
Adults present with cutaneous patches/nodules, indolent CD4+ mycosis fundgoides/Sezary syndrome
Bone pain plus anemia Multiple myeloma
What does CRAB stand for in multiple myeloma hyperCalcemia, Renal insuff, Anemia, Bone lytic lesions/Back pain
What is the age group most commonly affected by multiple myeloma 40 to 50
What is the characteristic lab finding on electrophoresis paraprotein spike, monoclonal protein
What are the main associations with multiple myeloma? inc suscept to infxn, primary amyloidosis, punched out lytic bone lesions, M spike, Ig light chains in urine, Rouleaud formations (stacked RBCs)
What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia? no lytic bone lesions in WM
Which cell is neoplastic in multiple myeloma plasma cell
What is monoclonal expansion without symptoms associated with multiple myeloma? MGUS - monoclonal gammopathy of undetermined significance
Upregulated growth of leukocytes in bone marro leukemia
Tdt+, CALLA+, kids in the marrow, adolescent males with mediastinal mass ALL
Where does ALL spread CNS and testis
What chromosomal translocation is associated with a better prognosis in ALL t(12;21)
can be asymptomatic, often in older adults, peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia CLL (SLL without the peripheral lymphocytosis
Elderly, mature b cell tumor with filamentous, hairlike projections Hairy cell leukemia
What does hairy cell leukemia stain with TRAP (tartrate resistant acid phosphatase
adults, auer rods, inc circulating myeblasts on peripheral smear AML
What is the therapy for the M3 variant? all trans retinoic acid
How does the therapy for M3 vairant work? induces differentiation of myeloblasts
philadelphia chromosome, blood looks like marrow CML
What is the philadelphia chromosome t(9;22) bcr abl
what is a blast crisis CML to AML or ALL
What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction low in CML
What is the therapy for CML (philly chrom) imatinib, anti bcr abl
What are the age ranges for the various leukemias ALL < 15, AML
What is makes a leukemia acute blasts > 5%
What do auer rods stain with peroxidase
What condition can result from treating AML M3 from the release of the Auer rods DIC
t(15;17) M3 AML (acute promyelocytic leukemia)
t(11:22) Ewing sarcoma
t(11;14) Mantle cell lymphoma
tennis rackets on EM Birbeck granules
S-100 and CD1a with birbeck granules Langerhans cell histiocytosis
abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors, inc RBC, JAK2 mut Polycythemia vera
Megakaryocytosis Essential thrombocytosis
Fibrotic obliteration of bone marow with teardrop cells Myelofibrosis (marow is crying being its fibrosed"
Does CML have a JAK2 mut no
What is relative polycythemia dec plasma volume
What is appropriate absolute polycythemia inc RBC, dec O2 sat, inc EPO
What is appropriate absolyte polycythemia associated with lung disease, congenital heart diseaes, and high altitude
What does ectopic EPO produce inappropriate absolute with inc RBCs and EPO
Wilm's tumor, RCC, HCC, hydronephrosis Polycythemia vera with inc plasma volume, inc RBCs, inc EPO
Created by: ykirti