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Hematology-Oncology Review Session. Pete Voorhees. Iron Deficiency Anemia. Symptoms of anemia (fatigue / weakness, SOB / DOE). Ice pica and koilonychia are specific for iron deficiency! Microcytic (low MCV), hypochromic (low MCHC) RBCs .

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iron deficiency anemia
Iron Deficiency Anemia
  • Symptoms of anemia (fatigue / weakness, SOB / DOE).
  • Ice pica and koilonychia are specific for iron deficiency!
  • Microcytic (low MCV), hypochromic (low MCHC) RBCs.
    • Other causes of microcytosis include thalassemias, sideroblastic anemias.
  • Ferritin is low, Serum Fe low, TIBC or transferrin normal or high, Fe saturation (serum Fe / TIBC) low.
  • Causes: chronic blood loss, malabsorption, decreased intake, pregnancy.
  • Treatment: Give iron, fix source of blood loss.
vitamin b12 deficiency
Vitamin B12 Deficiency
  • Symptoms of anemia.
  • Peripheral neuropathy (decreased proprioception, vibratory sense).
  • Macrocytic (high MCV) RBCs.
    • Other causes of macrocytic anemias include liver disease, folate deficiency, anemias ass. with a high retic. ct., hypothyroidism, HIV therapy (AZT), chemotherapy.
  • Hypersegmanted neutrophils.
  • Dx: low B12 levels.
  • Causes: pernicious anemia (Ab to IF), malabsorption (ileal resection), pancreatic insufficiency.
  • Treatment: replace B12.
folate deficiency
Folate Deficiency
  • Same symptoms as B12 deficiency but no neuropathy.
  • Macrocytic RBCs and hypersegmanted neutrophils.
  • Dx: low folate or RBC folate level.
  • Causes: Decreased intake (alcoholic), malabsorption, increased utilization (depletion) of body stores (chronic hemolytic anemia)
  • Treatment: Replace folate.
hereditary spherocytosis
Hereditary Spherocytosis
  • Symptoms of waxing / waning anemia, jaundice.
    • Hemolysis accelerated by infection.
  • Splenomegaly (hyperplasia secondary to increased workload), pigmented gallstones (h/o cholecystectomy), ankle ulcers.
  • Family history
    • AD. 1 : 5000 people of european descent affected.
hereditary spherocytosis1
Hereditary Spherocytosis
  • Blood smear: spherocytes, polychromatophilia (increased reticulocytes).
  • Labs: Increased retic. ct., increased LDH, increased indirect bilirubin, increased osmotic fragility.
  • Treatment: folate replacement, splenectomy in some circumstances).
  • Genetic defect: Spectrin, ankyrin mutations.
  • Pearl: Parvovirus B19 infection in patients with hemolytic anemis in general = aplastic crisis.
g6pd deficiency
G6PD Deficiency
  • Episodic hemolytic anemia.
    • Triggered by oxidant stress: drugs, infection.
  • Occurs in males
    • X-linked, 10 – 14% of males of African descent carry an unstable A- variant of G6PD.
  • More severe, chronic form seen in men of Mediterranean descent.
    • Think fava beans in a Mediterranean pt.
g6pd deficiency1
G6PD Deficiency
  • G6PD is required to generate NADPH and ultimately reduced glutathione.
  • Glutathione required to prevent oxidative damage to hemoglobin.
  • Deficient glutathione leads to oxidized, methemoglobin which precipitates out as Heinz bodies.
  • Macrophages of the RES phagocytose bits of RBC membrane with underlying precipitated hemoglobin.
g6pd deficiency2
G6PD Deficiency
  • Smear: Bite cells and blister cells.
  • Diagnosis: Smear, G6PD level, heinz body prep.
    • G6PD levels may be normal in the acute setting due to selective removal of older RBCs with lower baseline G6PD levels.
  • Treatment: Get rid of offending oxidant stress (drug, infection).
    • Important drugs to know that may precipitate hemolysis in these folks: SULFA, anti-malarial drugs, dapsone, vitamin K, fava beans.
warm autoimmune hemolytic anemia
Warm Autoimmune Hemolytic Anemia
  • Symptoms of anemia, jaundice, splenomegaly.
  • Smear: spherocytes, polychromatophilia.
  • Labs: Increased retic. ct., high LDH, high indirect bilirubin, + direct Coomb’s test (direct antiglobulin test or DAT).
    • Indirect Coomb’s usually positive as well.
  • Treatment: immune-suppression (steroids, spenectomy), treat / remove underlying trigger.
warm autoimmune hemolytic anemia1
Warm Autoimmune Hemolytic Anemia
  • Causes: Idiopathic, SLE, lymphoproliferative disorder (lymphoma, CLL).
  • Drugs
    • Innocent bystander: quinine, quinidine, INH
    • Hapten: PCNs, cephs
    • Autoimmune: alpha-methyldopa, procainamide
cold agglutinin disease
Cold Agglutinin Disease
  • Symptoms of anemia, acrocyanosis.
  • Smear, RBC agglutination, polychromatophilia.
  • Labs: Increased retic. ct., LDH, bilirubin, + Coomb’s test (C3 +. IgG -), + cold agglutinin titer.
  • Treatment: avoidance of cold, treat underlying disease, immune-suppression (chemotherapy).
  • Associated diseases: lymphoproliferative diseases (lymphoma, CLL) or after infectious mononucleosis or mycoplasma infection (“walking” pneumonia).
hemophilia a and b
Hemophilia A and B
  • X-linked.
  • Factor VIII (Hemo A) > Factor IX (Hemo B) deficiency.
  • Manifests as soft tissue and joint bleeds, provoked and spontaneous as well as other bleeding (intracranial, GU).
  • Long-term complications: Joint destruction from repeated bleeds, pseudotumors.
  • Labs: Prolonged aPTT, normal PT, normal TCT, normal platelet function screen and bleeding time.
  • Treatment: recombinant Factor VIII or IX replacement, ddAVP for mild hemophilia A (leads to release of endothelial stores of FVIII).
von willebrand s disease
Von Willebrand’s Disease
  • Autosomal dominant.
  • The most common inherited bleeding disorder.
  • Mucocutaneous bleeding (epistaxis, gum bleeding, GU/GI bleeding, menorrhagia).
  • Types 1 (mild deficiency) , 2 (qualitative abnormality), and 3 (severe deficiency).
  • Labs: Prolonged bleeding time / platelet function screen, slightly prolonged aPTT (due to low FVIII levels), low von Willebrand activity level, +/- low vWF antigen levels.
  • Treatment: Type 1: ddAVP. Type 2 and 3: vWF and FVIII-containing plasma product (Humate-P).
venous thrombosis
Venous Thrombosis
  • Causes
    • Acquired
      • Cancer
      • Myeloproliferative disorders (P. Vera, Essential thrombocytosis)
      • Antiphospholipid antibody syndrome
      • Hyperhomocysteinemia
      • Pregnancy
      • OCPs, HRT
      • Prior venous thrombosis
      • Age
      • Immobilization
      • Surgery
venous thrombosis1
Venous Thrombosis
  • Inherited causes
    • Factor V Leiden mutation!!!!
    • Prothrombin gene mutation
    • Protein C def.
    • Protein S. def.
    • Antithrombin def.
    • Dysfibrinogenemias, elevated FVIII, IX, XI levels
venous thrombosis2
Venous Thrombosis
  • Symptoms: pain / swelling in leg, chest pain, SOB (pulmonary embolism).
  • Diagnosis:
    • Duplex ultrasonography (doppler ultrasound)
    • IPG
    • Contrast venography
    • Magnetic resonance venography
    • D-dimer
venous thrombosis3
Venous Thrombosis
  • Treatment
    • Heparin or low-molecular weight heparin
      • Potentiates anticoagulant effect of endogenous anti-thrombin.
    • Warfarin
      • Depletes vitamin K-dependent coagulation factors (II, VII, IX, and X).
    • Fibrinolytics (tPA) if patient clinically unstable with extensive clot burden.
      • Activates the fibrinolytic enzyme, plasmin.
pseudothrombocytopenia
Pseudothrombocytopenia
  • Lab artifact!
  • The patient will have no bleeding history.
  • Clumps of platelets will be seen on the fringes of the smear.
  • Due to presence of EDTA in tube.
  • Diagnosis: smear, re-check plt count in citrated or heparin-anticoagulated tube.
disseminated intravascular coagulation
Disseminated Intravascular Coagulation
  • Diffuse, abnormal activation of coagulation, leading to consumption of clotting factors, and thrombocytopenia.
  • Clinically manifests as bleeding but the clinical picture is typically dominated by the disease that led to the DIC.
  • Prolonged PT, aPTT, TCT, and low platelets, low fibrinogen, low antithrombin, elevated D-dimer.
  • MAHA may be seen on the smear.
  • Causes: Severe infection, AML (esp. APL or M3 AML), obstetrical complications (eclampsia), severe burns.
  • Treatment: replacement (platelets, clotting factors with FFP, fibrinogen with cryoprecipitate), treat underlying disease.
thrombotic thrombocytopenic purpura ttp
Thrombotic Thrombocytopenic Purpura (TTP)
  • Abnormal activation of platelets and endothelium leading to fibrin deposition in the microvasculature and destruction of RBCs and consumption of platelets.
  • Pentad
    • MAHA
    • Thrombocytopenia
    • Fever
    • Renal failure
    • Neurologic deficits
  • Smear shows MAHA
  • Labs: PT, aPTT, TCT, fibrinogen, d-dimer are normal.
  • Cause: Primary (idiopathic) TTP due to autoantibodies to ADAMTS-13; secondary causes: pregnancy, drugs (mitomycin-C, quinine, ticlopidine, cyclosporine), HIV
  • Treatment
    • Plasma exchange
hemolytic uremic syndrome
Hemolytic Uremic Syndrome
  • Similar to TTP but renal failure dominates the clinical picture.
  • The blood smear will look the same and the lab work will be the same.
  • More common in children after diarrheal illness (esp. E. Coli O157/H7 and shigella).
  • Treatment: Supportive care +/- plasma exchange (less effective here than in TTP).
idiopathic thrombocytopenic purpura
Idiopathic Thrombocytopenic Purpura
  • The platelet equivalent of warm AIHA.
  • Symptoms: mucocutaneuos bleeding.
  • PE: petechiae.
  • Smear: absent / few platelets.
  • Causes: idiopathic, drugs (PCNs, sulfa (TMP-sulfamethoxazole, quinine), SLE, HIV, lymphoproliferative disorders.
    • Heparin causes an immune-mediated thrombocytopenia paradoxically associated with excessive clotting.
  • Treatment: Corticosteroids +/- IVIG, splenectomy for relapse, anti-D immune globulin, immunosuppressants.
polycythemia vera
Polycythemia Vera
  • Symptoms of increased viscosity: decreased mental acuity, blurred vision, tinnitus, headache, dizziness, paresthesias.
    • PV specific findings: post-bathing pruritus, erythromelalgia, thrombosis, hemorrhage, hypermetabolic symptoms.
  • PE: plethora, retinal vein distention, hepatosplenomegaly.
  • Labs: Increased WBCs, HCT, and platelets. Basophilia, high LAP score, high uric acid and vitamin B12, low erythropoietin level.
  • Treatment: phlebotomy, hydroxyurea, interferon-alpha, busulfan, P32.
    • Aspirin reduces the incidence of thrombosis.
essential thrombocytosis
Essential Thrombocytosis
  • Similar to P. Vera. Asymptomatic or excessive bleeding and / or clotting, splenomegaly.
  • Smear: large platelets. Labs: thrombocytosis, leukocytosis. Must r/o CML.
  • Treatment: age < 60, no clotting risk factors (smoking, HTN, etc.), plts < 1 – 1.5 million, no h/o clotting / bleeding – observation. Otherwise, hydroxyurea, anagrelide, or interferon-alpha.
    • ASA alleviates symptoms of microvascular occlusion (e.g.. erythromelalgia).
idiopathic myelofibrosis
Idiopathic Myelofibrosis
  • Symptoms of hypermetabolism (weight loss, fevers, sweats), splenomegaly (abd. pain, early satiety), anemia, +/- thrombocytopenia.
  • Leukoerythroblastic blood smear
    • Tear drop shaped RBCs, nucleated RBCs.
    • Left-shifted WBCs.
  • WBC count normal or high at diagnosis but eventually drops, HCT usually low at diagnosis, plts may be up, down or low.
  • Dry tap on bone marrow aspirate.
  • Increased fibrosis on bone marrow biopsy.
  • P. Vera and ET can evolve into a “spent,” myelofibrotic stage.
  • Treatment largely supportive, bone marrow transplant has been tried in younger patients.
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CML
  • Symptoms of hypermetabolism, splenomegaly, anemia.
  • Smear with increased numbers of WBCs (granulocytes of all stages of maturation).
  • Labs: Increased WBCs, +/- anemia, low LAP score, low vitamin B12 level.
  • Cytogenetics: t(9;22), BCR-ABL.
  • Treatment: Bone marrow transplant, Gleevec.
  • Monitoring disease: cytogenetics, FISH for t(9;22), PCR for BCR-ABL.
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