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Flashcards in Hematology Deck (59)
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What is acute lymphocytic leukemia (ALL)?

Child + lymphadenopathy + bone pain + bleeding + fever in child, bone marrow > 20% blasts in bone marrow
-population: children - most common childhood malignancy peak age 3-7
-highly responsive to chemotherapy (remission >90%)


What is chronic lymphocytic leukemia (CLL)?

middle age patient, often asymptomatic (seen on blood tests), fatigue, lymphadenopathy, splenomegaly
-population: adults - most common form of leukemia in adults - peak age 50 y/o
-diagnostic studies: SMUDGE CELLS on peripheral smear, mature lymphocytes
-treatment with observation, if lymphocytes are 100,000 or symptomatic, treat with chemotherapy


What is acute myeloid leukemia (AML)?

blasts + auer rods in adult patients
-population: adults (80%) majority of patient > 50 y/o
-anemia, thrombocytopenia, neutropenia, splenomegaly, gingival hyperplasia and leukostasis (WBC >100,000)
-aur rods and >20% blasts seen in bone marrow


What is chronic myeloid leukemia (CML)?

strikingly increased WBC count >100,000 +m hyperuricemia + adult patient (usually >50 years old)
-population: adults - patient usually > 50 y/o
-70% asymptomatic until the patient has a plastic crisis (acute leukemia)
-diagnostic studies: philadelphia chromosome (translation of chromosome 9 and 22) - "Philadelphia CreaM cheese" splenomegaly


What is anemia of chronic disease?

a form of anemia seen in chronic infection, chronic immune activation and malignancy
-multifactorial; often with iron deficiency


How is anemia of chronic disease dx?

usually needs the presence of chronic infection, inflammation, cancer, microcytic/normocytic anemia, and values for serum transferrin receptor and serum ferritin that are between those typical for iron deficiency and sideroblastic anemia


What are the lab values for anemia of chronic disease?

normal or decreased MCV, decreased TIBC, increased ferritin (high iron stores)
-some transferrin converted to lactoferrin which can't transport ferritin - decreased TIBC
-increased ferritin because it is not being transported to bone marrow


What are the MC causes of anemia of chronic disease?

chronic renal failures and anemia from connective tissue disorder: RA, SLE, HIV, CA, cirrhosis, chronic infection


What is anemia of renal failure?

erythropoiesis impaired because of decreased in EPO production and marrow responsiveness to EPO
-renal insufficiency + decreased serum EPO


How is anemia of renal failure dx?

normochormic/normocytic anemia initially, decresaed MCV, decrased TIBC, increased ferritin
-decreased serum erythropoietin in anemia of renal failure
-CBC and serum iron, ferriting, transferrin, transferrin receptro


What is the tx of anemia of renal failure?

treat underlying disorer, recombinant EPO and iron supplementation
-treat with EPO analog (Epogen, procrit) if Hgb < 10 and stop when Hgb >11 d/t increase chance MI/stroke


What are clotting factors?

are proteisn that interact to help the blood clot, stopping the bleeding
-there are two clotting factor disorders we should focus on
-von Willebrand Disese (vWD) - missing protein for platelt function
-the hemophilias
-hemophilia A decreased clotting factor VIII
-hemophilia B decreased clotting factor IX


What is von willebrand disease?

the von Willebrand factor is found in plasma, platelets, and walls of blood vessels
-when the factor is missing or defective, platelets cannot adhere to the vessel wall at the site of an injury
-as a result, bleeding does not stop as quickly as it should
-most common genetic bleeding disorder, autosomal dominant
-decreased von Willebrand's factor and decreased Factor VIII
-patients may present with excessive bleeding after a cute or increased menstrual bleeding
-you can differentiate this from hemophilia by lack of hemarthrosis, small amounts of superficial bleeding, common to have bleeding with minor injury and petechiae


How do you treat von willebrand disease?

treat with DDAVP (desmopressin) or in cases of excessive bleeding a transfusion of concentrated blood clotting factors containing von Willebrand factor


What is hemophilia?

a hereditary bleeding disorder caused by a deficiency in one of two blood clotting factors: factor VIII (A) or factor IX (B)


What are the two forms of hemophilia?

-hemophilia A, which accounts for about 80% of all cases, is a deficiency in clotting factor VIII ("aight")
-hemophilia B is a deficiency in clotting factor IX (Christmas disease)
-remember hemophilia A = "aight" and B comes after A which is factor nine
-x-linked recessive so will affect males (most of the time)
-hemarthrosis, bruising, and bleeding
-increased PTT, normal PT and platelets, with decreased factors VIII or decreased factor IX on assay


What is the tx for hemophilia?

treatment involves the replacement of factor VIII or IX


What is G6PD deficiency?

after infection or medication (oxidative stress) in an African American male (x-linked) + Heinz bodies and bite cells on smear (damaged hemoglobin - G6PD protects RBC membrane)
-hemolytic anemia
-african, middle eastern, s. asian population
-flare triggers: fava beans, antimalarials, sulfonamides
-diagnostic studies: Heinz bodies and bite cells on smear


What is the tx of G6PD deficiency?

-avoid potentially harmful drugs, monitor infection
-acute - blood transfusion


What is are the differential diagnosis of hypercoagulable state?

the differential diagnosis can be remembered with the mnemonic PVCs:
-platelets: too many (usually more than 1 million/uL) or overactive (TTP, heparin-induced thrombocytopenia (HIT), HUS, and HELLP)
-Vascular injury: from plows, trauma, or burns
-Clotting factors: anti-clotting factors protein C, protein S, or Antithrombin III deficient or not working
-Stasis and surgery


What is virchow triad?

blood stasis, hypercoagulable state, and vascular injury


What are the causes of hyper coagulable state?

-genetic causes include antithrombin III deficiency, factor V Leiden (activated protein C resistance), protein C deficiency, protein S deficiency, dysfibrinogenemia, and abnormal plasminogen
-acquired hypercoagulable states are associated with malignancy (Trousseua's syndrome), pregnancy, nephrotic syndrome, ingestion of certain medications (especially estrogen), immobilization, myeloproliferative disease, ulcerative colititis and Crohn disease, behcet syndrome, polycythemia vera, intravascular devices, DIC, hyperlipidemia (particularly familial type II hyperbetalipoproteinemia), PNH, TTP-HUS, hyperviscosity syndrome, anticardiolipin antibodies, HIT, and antiphospholipid syndrome
-heparin therapy (unfractionated heparin (UFH) or low-molecular-wegiht heparin (LMWH) can cause HIT, causing a decrease in platelts (usually in half), followed by platelet activation causing clotting and infarction
-lupus anticoagulant, an IgM or IgG immunoglobulin is seen in 5% to 10% of patients with SLE but is more common in persons without lupus or in those taking phenothiazines


What is Factor V Leiden (MC)?

procoagulant clotting factor - amplifies the production of thrombin - clot formation
-mutated factor V resistant to breakdown by activated Protein C - results in hypercoagulability
-symptoms/phycial exam - increased DVT and PE, especially in young patients
-diagnosis with activated protein C resistance assay (factor V Leiden specific functional assay) - if positive, confirm with DNA testing, normal PT/PTT
-tx: LMWH bridge to warfarin; long term antithrombotic therapy not recommended


What is protein C deficiency?

vitamin K dependent anticoagulant liver protein that stimulates fibrinolysis and clot lysis (inactivates factor V and VIII) - potentiated by protein S
-protein C deficiency causes an increased risk of recurrent DVT and PE, may have a family history
-dx: protein C/S functional assay: decreased protein C/S activity levels
-at risk for skin necrosis on warfarin
-tx: heparin and oral anticoagulation for life


What is protein S deficiency?

protein S is a vitamin K - dependent glycoprotein
-it serves as a cofactor for activated protein C, which inactivates procoagulant factors Va and VIIa, reducing thrombin generation
-venous thromboembolism (VTE; including deep vein thrombosis and pulmonary embolism) is the major clinical manifestation of protein S deficiency
-diagnose with protein C or S functional assay (preferred) - decreased protein C or S activity levels
-tx: heparin/oral anticoagulation for life


What is Antithrombin III Deficiency?

recurrent venous thrombosis and PE, repetitive intrauterine fetal death (IUFD)
-Antithrombin III = natural anticoagulant; inhibits thrombin (IIIa), Xa and other proteases; potentiated by Heparin
-associated with VTE; first episode 20-30 y/o
-tx: asymptomatic pt requires anticoagulation only before surgical procedures
-pt with thrombotic events require high dose IV heparin then oral anticoagulation indefinitely


What is Antiphospholipidi antibody syndrome?

autoimmune; often associated with SLE; characterized by thromboses and recurrent spontaneous abortions
-autoantibodies react against platelet membranes, activating endothelial cells and platelets - complement-mediated thrombosis
-dx: lupus anticoagulant, anticardiolipin, DRVVT test, prolonged PTT
-tx: high dose IV heparin with thrombotic events then oral anticoagulation indefinitely


What is idiopathic thrombocytopenic purpura (ITP)?

autoimmune reaction to platelets usually after a viral illness (ITP is insidious and chronic)


How is idiopathic thrombocytopenic purpura dx?

-associated with HIV, HCV, SLE, CLL
-CBC normal expect low platelets (+direct Coombs test)


What is the tx of idiopathic thrombocytopenic purpura?

-children supportive care (IVIG for refractory cases)
-adults treat with prednisone


What is iron deficiency?

decreased MCV (microcytic), decreased MCH (hypo chromic), increased TIBC, decreased ferritin (best test, low iron stores), target cells, pica, and nail spooning
-MC cause of anemia and usually from blood loss
-in men: chronic occult bleeding; women = menses
-always consider GI bleed
s/sx: fatigue, palpitations, SOB, weakness, HA, tinnitus (nail), atrophic glossitis (tongue), angular cheilitis
-associated with pica and nail spooning (kolionychia)


How is iron deficiency dx?

-microcytic/hypochromic anemia
-CBC: low reticulocyte count, high RDW
-iron studies: decreased serum iron, transferrin saturation; increased TIBC, decreased ferritin (best test, low iron stores) < 15 (diagnostic)
-H & H: <13.5 & 39 for men; <12 & 37 for women
-peripheral smear: poikilocytes; rarely bone marrow examination; hem occult if indicated


What is the tx for iron deficiency?

-ferrous sulfate 3 mg/kg once or twice daily between meals with juice (not milk)
-ferrous fumarate 100-200 mg/day in 2-3 doses; ferrous gluconate 3-6 mg/kg in 3 doses
-s/e: gray staining/teeth (liquid preps); GI upset/constipation
-six weeks to correct; six months to replete iron stores; recheck blood counts every 3 months x 1 year
-packed red blood cells when Hgb < 8
-other causes decreased MCV: lead poisoning, sideroblastic anemia, basophilic stippling, thalassemia


What is Hodgkin's disease Lymphoma?

HIGHLY CURABLE - painless lymphadenopathy + reed-sternbery + bimodal age (20s/50s) + B sx
-age: bimodal peaks in 20's then in 50's
-reed-sternberg cells ar pathognomonic - B cel proliferation with bilobed or multilobed nucleus "owl eye"
-painless lymphadenopathy: upper body lymp noes: neck, axilla, shoulder, chest (mediastinum)
-contigous spread to local lymph nodes: usually localized single group of nodes
-B symptoms are common - fever, weight loss, night sweats
-associated with EBV (40% of patients)
-Dx: CXR - mediastinal adenopathy
-tx: chemo, radiation, highly curable
-excellent 5-year cure rate (60%)


What is Non-Hodgkin's Lymphoma?

HIV patient + IG sx + painless LAD
->50 years old, increased risk with immunosuppression (ex HIV)
-B cells and T cells
-peripheral, multiple nodes; non-contiguous extranodal speard (GI and skin =MC)
-B symptoms are not common
-not associated with EBV
-s/sx: SOB, intussusception, bowel obstruction, abdominal masses
-tx: rituximab, chemo, variable course
-variable cure rate


What is multiple myeloma?

caner of monoclonal plasma cells; MC primary tumor of bone/bone marrow in pt > 50 yo
-older patient + bone and back pain refractory to treatments
-presentation: "CRAB" - calcium elevation, renal failure, anemia, and bone lesions


How is multiple myeloma dx?

-urinalysis: monoclonal proteinuria - Ig light chains (Bence jones protein)
-blood smear: rouleaux formation (stacked RBCs)
-radiograph: x-ray showing lytic "punched-out" bone lesions of skull, spine, long bones
-serum/urine electrophoresis: monoclonal (M protein) spike (IgG or IgA)
-bone marrow biopsy: >10% clonal plasma cells


What is the tx for multiple myeloma?

bone marrow transplant = definitive
-melphalan, steroids, thalidomide, bortezomib


What is sicke cell anemia?

African American, pain, family history of blood disorder, hemoglobin electrophoresis: hemoglobin S, blood smear: sickled RBCs, Howell-Jolly bodies, target cells
-chronic hemolytic anemia occurs almost exclusively in blacks
-hemolysis, jaundice, splenomegaly, priapism, poor healing, pain/swelling hands and feet, acute chest syndrome, pigmented gallstones
-sickle-shaped RBCs clog capillaries causing organ ischemia (crises)
-a very high reticulocyte count can be used to monitor event (decreases as pt improves)


How is sickle cell anemia dx?

diagnosis requires (+) Hgbs on hemoglobin electrophoresis - is definitive
-reticulocytosis; mildly increased WBCs
-blood smear: sickled RBCs, howell-jolly bodies, target cells
-Hgb 8-10, HCT 20-30
-RBcs = normochormic, normocytic
-Hb SS = disease
-Hb SA = trait
-two parents with sickle cell trait have a 1 in 4 chance of having a child with Hb SS disease


What is the tx of sickle cel anemia?

high flow O2, pain control during a crisis, supportive transfustion when Hgb < 6
-vaccines (meningococcal, pneumococcal, H. influenzae, influenza), prophylactic antibiotics, and aggressive treatment of infections prolong survival
-hydroxyura may decrease the frequency of crisis
-infection, bone marrow aplasis, lung involvement can develop acutely and be fatal


What is thalassemia?

family history of blood cell disorder, microcytic hypo chromic, elevated iron


What is beta thalassemia major?

-most severe, mediterranean descent, failure to thrive
-hemoglobin electrophoresis: hemoglobin A2 and F
-treatment: transfusion dependent, iron chelation (deferoxamine)


What is beta thalassemia trait?

-mild anemia, often misdiagnosed as iron deficient
-hemoglobin electrophoresis: hemoglobin A2


What is alpha thalassemia?

-chinese and southeast asians
-hemoglobin electrophoresis: hemoglobin H (H disease), hemoglobin Bart's (hydros fettles), hemoglobin A (trait)


What is TTP vs ITP?

different from ITP (ITP is insidious and chronic) from TTP which is an acute febriel disease with multi-organ thrombosis (hence the name "thrombotic" thrombocytopenia)


What is thrombotic thrombocytopenia (TTP)?

decreased platelets + anemia + schistocytes (RBC fragments) on smear


What is the caue of thrombotic thrombocytopenia?

-after drugs: quinidine, cyclosporine & pregnancy
-inhibition of ADAMTS13


What is the presentation of thrombotic thrombocytopenia?

-purpura and "FAT RN" - fever, anemia, thrombocytopenia, renal failure, neurological sympotms


What are the diagnostic stuides of thrombotic thrombocytopenia?

CBC normal expect low platelets, schistocytes (RBC fragments) on the smear (-) Coombs test


What is the treatment of thrombotic thrombocytopenia?

steroids, plasmapheresis


What is hemolytic uremic syndrome (HUS)?

decreased platelets + anemia + renal failure (associated with E. coli o157: H7 and diarrheal illness in a child)


What is the presentation of hemolytic uremic syndrome?

-post-infection: E. coli or shigella
-severe kidney problems


What is Vitamin B12 deficiency?

increased MCV > 100 (macrocytic anemia), hyperhsegmented neutrophils and normal folate, decreased vibratory and positive sense


What is the the etiology of vitamin B12 deficiency?

pernicious anemia (antibody to intrinsic factor), gastrectomy, vegans


What are the characteristics of vitamin B12 deficiency?

-glossitis: smooth beefy, sore tongue, neurologic symptoms (poor balance, low proprioception)
-diagnostic studies:
-megaloblastic anemia (MCV >100), hyperhsegmented neutrophils
-elevated serum MMA, elevated homocysteine
-pernicious anemia: schilling test (less than 10% radio labeled vitamin B12in urine, normal results when repeated with the administration of intrinsic factor)


What is the tx of Vitamin B12 deficiency?

lifelong IM B12: 1-3 ug/d (animal products, fortified cereal) for pernicious anemia
-IV cyanocobalamin 1 mg IM daily x 7 d, then weekly x 4 wk, then monthly for life
-PO B12 1-2 mg PO daily for vegans and bariatric surgery
-years to deplete stores


What is folate deficiency?

decrease folate, increased MCV (macrocytic anemia) - looks like. B12 but no neurologic symptoms
-population: alcoholics
-diagnostic studies:
-megaloblastic anemia
-serum folic acid: low
-macro-ovalocytes and hypersegmented PMNs (pathognomonic)
-elevated homocysteine, normal MMA


What is the tx of folate deficiency?

-PO folic acid 1-5 mg/d (first line)
-avoid ETOH and folic acid antagonists (Bactrim, phenytoin, sulfasalazine)
-green leafy vegetables, yeast, legumes, fruits, animal proteins
-prophylactic folic acid - pregnant/lactating women, contemplating pregnancy, sickle cell patient