Hematology oncology review session
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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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Hematology-Oncology Review Session

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Hematology oncology review session

Hematology-Oncology Review Session

Pete Voorhees


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.


Hematology oncology review session

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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