Flashcards in Hem Onc Deck (116):
Iron deficiency/thalassemia/anemia chronic disease iron, tibc, ferritin?
Iron deficiency-decreased iron, ferritin, increased TIBC, decrease transferrin
Thalassemia-increased iron, ferritin, decreased TIBC, increased transferrin
ACD-decreased iron, decreased TIBC, normal/increased ferritin, normal/decreased TIBC
Triad of symptoms in lead poisoning?
GI (abdominal pain, constipation), Neuro (peripheral neruopathy, cognitive impairment), Anemia (microcytic)
Tx. and complications of hereditary spherocytosis
Tx. folic acid, blood transfusions, splenectomy (definitive). Complications are pigment gallstones (extravascular hemolysis), aplastic crisis from B19 infection
Lab findings G6PD (LDH, haptoglobin, Hb, indirect bilirubin)
Increased LDH, decreased haptoglobin, decreased Hb, increased indirect bilirubin
Lab findings PNH?
Pancytopenia (destruction of all cells due to deficit of CD55/59), Elevated LDH and low haptoglobin, unconjugated hyperbilirubinemia, hemoglobinuria
-Eculizumab (monoconal Ab inhibiting complement activation)
HUS findings? (4)
Microangiopathic hemolytic anemia (w/thrombocytopenia), fever, skin and mucosal bleeding, renal insufficiency (increased creatinine, hematuria, proteinuria, casts)
VTE testing for patients with more concerning findings of malignancy or recurrent or multiple site (eg cerebral, hepatic vein)
CT scan abdomen/pelvis/chest
Giant cell tumor (location, x-ray finding, pathogenesis)
Epiphysis, soap-bubble, osteolytic lesions from large osteoclast giant cells
Lymphadenopathy (benign vs malignant) characteristics
benign-2 cm, firm, solitary
Androgen abuse reproductive, CV, psychiatric and hematologic consequences
Reproductive (gynecomastia, small testes, azospermia), CV(LVH, increaed LDL and low HDL), Aggression and mood problems, hematologic (polycythemia and possible hypercoagulability)
MGUS vs Multiple Myeloma
MGUS (no CRAB, monoclonal protein 3 g/dL, >10% plasma, B2 microglobulin increased)
ITP lab finding/peripheral smear?
Lab finding: isolated thrombocytopenia, megakaryocytes on peripheral smear
ITP treatment children
Skin manifestations only: observe; bleeding: IVIG/glucocorticoids
ITP treatment adults
>30K without bleeding: Observe;
Intravascular vs Extravascular hemolysis pathogenesis
Intravascular-RBC structural damage leading to RBC structural damage in intravascular space
Extravascular-RBC destroyed by phagocytes and RAA
Tx. AIHA (warm agglutinin vs. cold)
Warm-corticosteroids, IVIG, splenectomy for refractory
Cold-avoid cold, Rituximab +/- fludarabine or cyclophosphamide, cyclosporine, or other immunosuppresive stop production of antibody. STEROIDS DO NOT WORK COLD AGGLUTININ
Risk of sepsis for how long after splenectomy and recommendations to help try and decrease risk?
30 yrs or longer, triple vaccine 3-5 wks before splenectomy and oral penicillin for 5 years after
Features, timeframe, and cause of transfusion-related acute lung injury?
Dyspnea and pulmonary edema, within 6 hours, caused by donor anti-leukocyte antibodies
What is primary hypotension reaction and where is it seen?
Hypotension often seen in patients taking ACE inhibitors. Occurs within minutes of transfusion and caused by bradykinin in blood products as well as normal bradykinin buildup in patients
Triad of osler weber rendu?
Widespread telangiectasias, recurrent eistaxis, widespread AV malformations (mucus membranes, skin, GI tract)
Tx. of cancer related anorexia/cachexia syndrome?
Progesterone analogs (megestrol acetate and medroxyprogesterone acetate) and corticosteroids
Which 3 patient populations need irradiation to RBC before transfusion?
1) BMT recipients
2) Acquired or congenital cellular immunodeficiency
3) Blood components donated by 1st/2nd degree relatives
Which 4 patient populations need leukoreduction before RBC transfusion?
1) Chronically transfused patients (eg hemochromatosis)
2) CMV seroneative at risk patients (eg aids, transpant patients)
3) Potential transplant recipients
4) previous febrile nonhemolytic transfusion rxn
Which 3 populations need washing before RBC transfusion?
1) IgA deficieny
2) Complement dependent autoimmune hemolytic anemia
3) continued allergic rxn despite antihistaine
Anaphylaxis symptoms, timeframe, cause?
Urticaria, hypotension, dyspnea, angioedema, seconds to minutes, recipient IgA antibodies
Febrile acute hemolytic symptoms, timeframe, cause?
Fever, chill, flank pain, hemoglobinuria, renal failure and DIC.
Febrile nonhemolytic symptoms, timeframe, cause?
Fever and chill, 1-6 hours, cytokines accumulation during blood storage
TRALI (transfusion associated acute lung injury) symptoms, timefram, cause
Dyspnea, non-cardiogenic pulm edema, within 6 hours, donor anti-leukocyte antibodies
Allergic rxn blood transfusion symptoms, timeframe, cause?
Urticaria, flushing, angioedema, pruritis, 2-3 hours of transfusion, recipient IgE antibodies and mast cell activation
Delayed hemolytic symptoms, timeframe, cause?
Mild fever and hemolytic anemia, 2-10 days after, anamnestic antibody response
Gold standard confirmatory test HIT?
Serotonin release assay
Remove all heparin. Supplement with fondaparinux or DTI (eg agratroban)
Factor Xa vs Warfarin (MOA, therapeutic effect, acute dvt tx, need for monitoring, antidote for hemorrhage)
Xa: DTI, 2-4 hrs, single dose, no monitoring, no antidote)
Warfarin: vit k antagonist, 5-7 days, bridge with heparin for 5 days, PT/INR, ffp and vit K as antidote)
Initial drug for hypercalcemia of malignancy depending on calcium level?
Asymptomatic or 14): Acute Normal saline plus calcitonin and long term with bisphosphonates
Anemia cause best initial test?
Anemia cause next best test?
Peripheral blood smear
Echinocytes or burr cells on smear etiology?
Where is folate deficiency commonly seen?
Alcoholics and pregnant women
MCC B12 deficiency
Confirmation of B12 deficiency
Tx of Bthal major or minor?
B thal minor-generally no treatment. B thal major-transfusions, iron chelation therapy to prevent secondary hemochromatosis.
4 commonly tested causes of autoimmune hemolytic anemia?
-Lupus erythematous (lupus like syndromes, such as procainamide, hydralazine, and isoniazid)
-Drugs (methyldopa, but penicillins, cephalosporins, sulfa drugs, and quinidine have also been implicated)
-Leukemia or lymphoma
-Infection (mycoplasma, EBV, syphilis)
When should screening be performed for children with lead poisoning?
6 months in children with risk factors
Tx. lead poisoning
avoid exposure (best strategy) as well as lead chelation if necessary. Use succimer in children and dimercaprol in adults; in severe cases, use dimercaprol plus EDTA for children/adults
Tx for sideroblastic anemia
What is myelophthisic anemia? Waht clues on smear/biopsy suggest its presence?
-Space occupying lesion in bone marrow. common causes are malignant invasion that destroys bone marrow and myelodysplasia or myelofibrosis
-Smear with anisocytosis (diff size), poikilocytosis (diff shape), nucleated RBC, giant and/or bizarre looking platelets and teardrop shaped RBC
-Biopsy either "dry tap" or malignant cells
MCC blood transfusion rxn
Lab error. Use O neg blood to avoid a rxn when you can't wait for blood typing or when blood bank does not have patient's blood type
Side effect large transfusions?
Bleeding diathesis from dilutional thrombocytopenia and citrate as calcium chelator
Best initial therapy/ Long term therapy for waldenstrom macroglobulinemia?
Long term-rituximab or prednisone or cyclophosphamide
Treat underlying condition followed by FFP
Deficiency of ADAMTS 12 (vWF metalloprotease)-->degradation of vWF multimers--> increase large vWF multimers-->increase platelet adhesion-->increase platelet aggregation and thrombosis
Fever, thrombocytopenia, hemolytic anemia, neurologic sequale
Plasmapheresis, steroids. NO PLATELET b/c WORSE DISEASE
3 major drugs that cause folic acid deficiency and subsequent anemia that need supplementation?
Phenytoin, Methotrexate (vs 5-FU not reversible with folic acid), Trimethoprim
Expected symptoms with certain hematocrit
>30-35, 25-30, 20-25,
None, Dyspnea (worse on exertion) and fatigue, Lightheadedness and angina, syncope and chest pain
Reticulocyte count in microcytic anemia? What is exception?
Low reticulocyte count. Alpha thal with 3 gene deleted (HbH) has reticulocytosis
Reticulocyte count in macrocytic anemia?
When is reticulocyte count high?
Blood loss and hemolysis
Indicate to transfuse (PRBC) for anemia?
Symptomatic, elder patient with heart disease and low hematocrit (
Indication for FFP?
DIC, warfarin reversal, cirrhosis
MCC sideroblastic anemia?
Alcohol-->bone marrow suppression
Best initital test microcytic?
Only form of microcytic anemia where circulating iron is elevated?
Most accurate test sideroblastic?
Most accurate test iron deficiency?
Bone marrow biopsy for sustained iron
Most accurate test thalassemia and findings?
Electrophoresis. Findings: Alpha normal until 3 gene deleted with HbH and 4 gene deleted in Hb bart (incompatible with life). B-minor (HbA2>3.5%), B-major (increase HbF+A2 on electrophoresis)
Microcytic Anemia with ESRD routinely responds to what?
Erythropoietin replacement. Must be careful and have adequate iron stores because risk of EPO induced surge in RBC production
Where is B12 absorbed?
Increase homocysteine and MVA in what deficiency compared to increased homocysteine in what deficiency?
Complication of B12 or folate replacement?
Best initial/most accurate test sickle cell?
Peripheral smear/Hb electrophoresis
Tx. sickle cell
Hydration/oxygen, fever or elevated WBC use abx (ceftriaxone, levofloxacin or moxifloxacin), folic acid (excess cell turnover and reticulocytosis) replacement, hydroxyurea
When is exchange transfusion indicated for severe vasoocclusive crisis?
-Acute chest syndrome, priapism stroke, visual disturbance from retinal infarction
What is aplastic crisis in sickle cell? Best initial/most accurate test and best therapy?
Parvovirus B19 infection that freezes bone marrow production decreasing reticulocyte count.
Best initial-CBC, most accurate-PCR for DNA. IVIG for therapy
Most accurate test AIHA warm vs. cold?
Coombs test (warm), Cold agglutinin titer (cold)
Best initial/most accurate G6PD?
Best initial-PBC (bite cell and heinz body w/ methylene blue), Most accurate-g6pd 1-2 months after onset of hemolysis
HUS vs. TTP associations for cause?
HUS-E coli O157:H7
TTP-ticlodipine, clopidogrel, cyclosporine, AIDS, SLE
Presentation and etiology aplastic anemia?
Anemia, infection, thrombocytopenia (pancytopenia) of unclear etiology
Definitive tx aplastic anemia?
50: Antithymocyte globulin (ATG) and cyclosporine/tacrolimus
Why pruritis in PC vera?
Histamine release from increased basophils
Most accurate test PC vera?
JAK2 mutation (V617F)
Tx. PC vera?
Phlebotomy and aspirin prevent thrombosis, hydroxyurea helps lower cell count, allopurinol or rasburicase protects against uric acid rise, antihistamines
What drug inhibits JAK2?
Essential thrombocytosis vs reactive thrombocytosis?
Essential greater than 1 million, Reactive 500-700k
Essential thrombocytosis tx.?
60 w/ thromboses or >1.5 million: Hydroxyurea or anagrelide (when red cell suppression from hydroxyurea)
Tx for myelofibrosis?
55: Thalidomide/lenalidomide (increase bone marrow production)
Best initial/most accurate acute leukemia?
-PBC showing blasts best initial, flow cytometry most accurate
Most common presentation AML (M3 type)?
Best initial/most accurate acute leukemia?
Best initial-PBC, most accurate-flow cytometry
Translocation AML and ALL?
AML-(15;17), ALL (12;21) is good prognosis or (9;22) is bad prognosis
2) Poor prognosis (via cytogenetics)--BMT; Good prognosis-->More chemo
3) Add ATRA (AML) or intrathecal methotrexate (prevent CNS/testes metastases) for ALL
Best initial/Most accurate test CML
1) LAP, 2) BCRABL on PCR or FISH peripheral smear.
Tyrosine kinase inhibitors (imatinib) best initial and BMT curative
WHO CML phases?
Chronic -20$ blasts with extramedullary hematopoiesis
Characteristics of myelodysplastic syndrome?
Hypercellular bone marrow with megaloblastic hematopoiesis, pancytopenia. Risk of transformation to AML but most die from infection/bleeding before. Blasts
Characteristic cell of myelodysplastic syndrome>
Pelger Huet (bilobed nucleus)
Tx. myelodysplastic syndrome
1) Transfusion 2) Erythropoietin 3) Azacitidine or decitabine 4) Lenalidomide (for those with 5q deletion) 5) BMT
Increased WBC, increased lymphocytes, smudge cells in ELDERLY.
What is richter phenomenon?
Occurs in CLL where conversion to high-grade lymphoma happens in 5% of patients
Tx based on staging CLL?
Stage 0 (elevated WBC), Stage I (lymphadenopathy), and stage II (hepatosplenomegaly) there is no Treatment! Stage III (anemia) and stage IV (thrombocytopenia) are treated with fludarabine, cyclophosphamide, AND rituximab. If fludarabine fails, Alemtuzumab (anti-CD-52)
Tx. hairy cell?
Cladirabine or pentostatin
Best initial/most accurate hairy cell?
Best initial-Smear w/ hairy cells
Most accurate-immunotyping by flow cytometry (CD11c) or TRAP +
Best initial test non-hodgkin lymphoma
Tx. local vs advanced disease non-hodgkin lymphoma>
Local (stage 1a and IIa)-radiation and small dose/course chemo
Advanced (stage III/IV and B symptoms)-combination chemo with CHOP (Cyclophosphamide, adriamycin, vincriostine (oncovin), prednisone) and rituximab, antibody against CD20
Tx. hodgkin disease?
Stage Ia and IIA: local radiation with small dose chemo
Stage III/IV or any B symptoms: ABVD (adriamycin, bleomycin, vinblastine, dacarbazine)
Vincristine side effect?
Cisplatin side effect?
Most common presentation/cause of death in multiple myeloma?
Presentation-pathologic bone fracture
Cause of death-Infection followed by renal failure
Best initial most accurate multiple myeloma?
Best initial-x-ray of bone. Most accurate: Plasma cell>10% on bone marrow biopsy in addition to SPEP (spike) and bence jones protein
Best initial-combo dexamethasone with lenalidomide, bortezomib, or both
von willebrand disease tx?
desmopression and use factor VIII or vWF concentrate with no response
Factor XI deficiency tx?
No bleeding-no therapy
Factor XII deficiency tx?
No bleeding so no therapy needed
FFP, cryopercipitate if FFP NOT control bleeding