Hematology - Oncology emergencies

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<p>Hem-Onc Emergencies</p> <p>Hematology Emergencies Akshat Jain ,PL6Fellow Boot Camp orientation7/3/14 List of common emergencies Platelet emergencies </p> <p>Sickle cell emergencies </p> <p>The bleeding hemophiliac </p> <p>Hyperbilirubinemia in Neonate </p> <p>TEC </p> <p>BloodSuspension of cells in a solute of water, proteins, and electrolytes</p> <p>Average volume is 5 liters70mL per kg body weight</p> <p>PlasmaBlood from which the cellular components Color is yellowContains coagulation proteins (clotting factors)</p> <p>Hematopoiesis</p> <p>Hematologic and Immunologic EmergenciesMay present with severe illness, shock. Special considerations include:SepsisAcute chest syndrome with sickle cell crisisStroke with sickle cell crisisTumor lysis syndrome Increased overall risk of infectionHematologic and Immunologic EmergenciesQuickly assess for signs of sepsis and decompensation.Examination should include:Lung, circulatory, and neurologic examinationEvaluation of the extremities for swollen joints Hematologic and Immunologic EmergenciesBecause some patients have indwelling catheters, evaluate catheter site for:ErythemaSwellingTenderness Can be signs of central line infectionsAbnormal BleedingEpistaxis unrelieved by 15 minutes of pressure, both nostrils, requiring an ER visit, documented drop of Hb. </p> <p>Menstrual periods( amount, pads, duration)</p> <p> Bleeding after procedures (circumcision, dental extractions, T and A-delayed bleed)</p> <p>Ecchymoses/bruising inconsistent with the degree of trauma</p> <p>8Causes of Bleeding - Coagulopathy:Primary:vWF DeficiencyHemophiliaPlatelet dysfunctionSecondary:DICAnticoagulantsVit K deficiencyHepatic FailureRenal FailureMaternal Anticonvulsant</p> <p>Causes of BleedingVascular(Non-Hematologic)Child AbuseVasculitisUlcerVaricesEhlers-Danlos SyndromeTelangiectasiaAngiodysplasia</p> <p>CharacteristicPrimary hemostasisSecondary hemostasisOnsetSpontaneous and immediateDelayed after traumaUsual siteSkin, mucous membranesDeep tissues / hemarthrosisOther sitesRareRetroperitoneum, CNSExamplesThrombocytopenia, platelet defects (vWD)Factor deficiency or inhibitorCase with Bleeding ? Think - Patterns of bleedingPrimary hemostasis platelet and vascular functionSecondary hemostasis clotting factors</p> <p>CNS bleeding is leading cause of death among hemophilia patients12Platelet emergencies Neonatal thrombocytopenia </p> <p>Immune thrombocytopenia Newborn-ThrombocytopeniaA newborn has a completely normal physical exam except for a few petichiae. Platelet 50,000.</p> <p>14Immune thrombocytopeniaAuto-immune: Pregnant women with ITP/Hx of ITP </p> <p>Passive transfer of antibodies (IgG) from mother. Even when mother has a normal platelet count (Splenectomy)Nadir-few days; Platelets &lt; 50,00 have 1% risk of ICH.IVIG to mother, Fetal platelet counts, C sec, US, IVGG to baby</p> <p>Iso-Immune: Normal platelet count in mother</p> <p>Similar to Rh disease; PL A1 antigen/ Zw a negative mother.97% of population is PL A 1 positiveSensitization early in pregnancyPlt function defect because Anti-PL A1 interferes w/aggregation.Severe bleeding more likely; first born affected; Recovery in 2-3 weeksMothers washed (PLA1 neg) platelets; IVIG; Ultrasound; Steroids15Signs </p> <p>16Kasabach-Merritt , TAR</p> <p>17Case 10 year old male treated with Valproic acid for seizures presents with fever. He appears Ok with no skin lesions, lymphadenopathy or hepatosplenomegaly.Labs - CBC WBC 5, Hb 12, Platelet 65,000. BUN 12, Creatinine 0.6 md/dl.</p> <p>What is the Most likely cause:ITP (Immune thrombocytopenic purpura)HUS (Hemolytic Uremic Syndrome)HS Purpura (Henoch- Schonlein Purpura)ALL (Acute lymphoblastic leukemia)Drug induced purpura18Other causesHemolytic Uremic Syndrome Hemolysis, sick patient, Uremia, microangiopathicHenoch-Schonlein Purpura Purpuric lesions on lower extremities and buttocksAbd pain, arthritis. IgA depositionALL lymphadenopathy (LN), hepatosplenomegaly,other cell lines affectedDrug induced- LikelyBy reducing production or increasing destruction</p> <p>19Whats different ? </p> <p>ITP</p> <p>ITPUsually acute onset; immune mediated; post viralPeak 2-5 years of age, males=femalesSpontaneous bruises, petechiaePE no lymphadenopathy (LN), hepatosplenomegaly.CBC- other cell lines normal, large plts on smearTreat if plt&lt; 10,000 or wet ITP, avoid NSAIDS, AspirinTreat- IVIG best response, 48-72 hours; Side effects.Anti-D (WInRho) Rh+ ,hemolysis, quick responseSteroids good response, SE, inexpensive, need BMBM- Increased megakaryocytes, otherwise normal22Win Rho and Monoclonal ab.</p> <p>In untreated patients with this disease, IgG antiplatelet antibodies promote destruction of platelets, because of clearance by splenic macrophages. (B) After treatment with anti-RhoD immunoglobulins, the opsonized erythrocytes that are cleared saturate the clearance or phagocytic capacity of splenic macrophages, and IgG-coated platelets are spared. (C) Similarly, when patients are treated with anti-CD20 antibodies (rituximab), opsonized B cells saturate clearance mediated by splenic macrophages, and IgG-coated platelets are again spared.23Bleeding DisordersCoagulation cascade</p> <p>Bleeding Hemophiliac </p> <p>The bleeding hemophiliacThe known hemophiliac in the ED - RED BINDER !!! </p> <p>Hemophilia -A (classic): factor 8 deficiency B (christmas disease): factor 9 deficiencyBoth coded on X chromosomeDisease due to absent or dysfunctional proteinClinically INDISTINGUISHABLEPrevalence: 1 in 7500 malesHemophilia A seven times more common than hemophilia B</p> <p>The bleeding hemophiliacGrading according to degree of factor dysfunctionality:severe: 5% native activity</p> <p>Where do hemophiliacs bleed? Everywhere!!!Mucocutaneous bleeds (epistaxis , oral, GI)Soft Tissue bleeds Hemarthoses Muscle bleeds (ileopsoas!)CNS The bleeding hemophiliacRx - Respective recombinant factor replacement (both F8 and F9 deficiency)DDAVP (mild factor 8 deficiency)Antifibrinolytics (amicar)</p> <p>Life threatening Emergencies FFP **** (factor 9 deficiency)cryoprecipitate **** (factor 8 deficiency)Managing the bleeding hemophiliacDOSING SPECIFICS</p> <p>Recombinant factor 8 dosing:1 unit/kg of factor 8 raises the activity level 2%</p> <p>Recombinant factor 9 dosing:1.5 unit/kg of r-factor 9 raises the acvity level 1%Beware anaphylaxis in F9 replacement</p> <p>FUNDAMENTAL PRINCIPLE:when in doubt, treat!!!!</p> <p>Standard rule of thumb:correct the bleeding hemophiliac child to 80% - 100% activityRefractory hemophiliac in the ED Barriers to therapy: the dreaded factor inhibitor25% - 30% in F8 deficiency3% in F9 deficiency</p> <p>Patient with inhibitor in ED Bypassing agents FEIBARecombinant VIIa (novoseven)Antifibrinolytics (amicar)AnemiaDefined by measurement of hemoglobin concentrationNormal 15 in males ; 14 in femalesPatients are anemic when hgb is &gt; 2 standard deviations below normal Determining reticulocyte count and MCV are first steps in determining etiology</p> <p>Almost 1/3 of the world population is anemic!</p> <p>Increase in hgb that occurs in men is mostly related to effect of androgens on release of erythropoietin ; gender disparity becomes less significant in advanced age31Hemolytic AnemiaHistory and physical findingsReview of PMH, FH, and medicationsJaundice is commonOccasional LUQ abdominal discomfort (splenomegaly)</p> <p>Lab findingsElevated reticulocyte count Reflects bone marrow compensating for peripheral RBC destructionElevated LDH Elevated total bilirubin (indirect/unconjugated)Decreased haptoglobinAbnormal cells on peripheral blood smear examination</p> <p>What's Wrong ?</p> <p>What's Wrong ? </p> <p>Sickle cell emergencies </p> <p>Hgb S gene carried by 8% of African Americans1:625 have disease</p> <p>35Sickle Cell Disease (SCD)Genetically inherited autosomal-recessive disorder of red blood cellsResults in abnormal sickling of the red blood cells resulting in occlusion ( Gel Sol transformation defect )Leads to ischemia and painful crisesSickle Cell Disease (SCD)Infants may present with: FussinessIrritabilityCryingPoor feedingNonspecific findingsOlder children may report: Pain in specific locations, including joints, back, and chestSickle cell crisesVaso-occlusive crisis-dactylitis, long bones, back, chest. Trt. Pain meds, hydration.Aplastic crisis: low Hb, low retic, Sec to Parvovirus infection.Splenic sequestration crisis: spleen palpationHyperhemolytic crisis</p> <p>38Sickle Cell Disease (SCD)General principles : Gentle hydrationAnti-inflammatory medications and narcotics .STAT CBC w Reticulocyte count , Type and cross.Supplemental oxygen ( controversial ) .</p> <p>STROKE Can be ischemic (younger) or hemorrhagic (older ) .</p> <p>Administer oxygen and IV fluids .CBC w retic, Type and cross .CT Scan brain .Alert Blood bank STAT - exchange transfusion .</p> <p>Case12 year old female with SS disease complains of right sided chest pain and upper back pain for one day. P/E reveals slightly reduced breath sounds and a Pulse OX of 86 %. CXR shows an infiltrate on the right lower lobe.What is your diagnosis?What will you do next?41ACS</p> <p>Gladwin MT, Vichinsky E. N Engl J Med 2008;359:2254-2265.Definition- Diagnostic criteria for ACSACS is defined as radiographic evidence of consolidation: a new segmental (involving at least one complete segment) radiographic pulmonary infiltrate,ANDat least one of the following:</p> <p>Temperature 38.5C&gt;2 percent decrease in SpO2(O2saturation) from a documented steady-state value on room air (FiO2= 0.21)PaO2 </p>