hematology - oncology emergencies
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Hematology Emergencies Akshat Jain ,PL6Fellow Boot Camp orientation7/3/14 List of common emergencies Platelet emergencies
Sickle cell emergencies
The bleeding hemophiliac
Hyperbilirubinemia in Neonate
BloodSuspension of cells in a solute of water, proteins, and electrolytes
Average volume is 5 liters70mL per kg body weight
PlasmaBlood from which the cellular components Color is yellowContains coagulation proteins (clotting factors)
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.
Menstrual periods( amount, pads, duration)
Bleeding after procedures (circumcision, dental extractions, T and A-delayed bleed)
Ecchymoses/bruising inconsistent with the degree of trauma
8Causes of Bleeding - Coagulopathy:Primary:vWF DeficiencyHemophiliaPlatelet dysfunctionSecondary:DICAnticoagulantsVit K deficiencyHepatic FailureRenal FailureMaternal Anticonvulsant
Causes of BleedingVascular(Non-Hematologic)Child AbuseVasculitisUlcerVaricesEhlers-Danlos SyndromeTelangiectasiaAngiodysplasia
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
CNS bleeding is leading cause of death among hemophilia patients12Platelet emergencies Neonatal thrombocytopenia
Immune thrombocytopenia Newborn-ThrombocytopeniaA newborn has a completely normal physical exam except for a few petichiae. Platelet 50,000.
14Immune thrombocytopeniaAuto-immune: Pregnant women with ITP/Hx of ITP
Passive transfer of antibodies (IgG) from mother. Even when mother has a normal platelet count (Splenectomy)Nadir-few days; Platelets < 50,00 have 1% risk of ICH.IVIG to mother, Fetal platelet counts, C sec, US, IVGG to baby
Iso-Immune: Normal platelet count in mother
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
16Kasabach-Merritt , TAR
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.
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
19Whats different ?
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< 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.
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
The bleeding hemophiliacThe known hemophiliac in the ED - RED BINDER !!!
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
The bleeding hemophiliacGrading according to degree of factor dysfunctionality:severe: 5% native activity
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)
Life threatening Emergencies FFP **** (factor 9 deficiency)cryoprecipitate **** (factor 8 deficiency)Managing the bleeding hemophiliacDOSING SPECIFICS
Recombinant factor 8 dosing:1 unit/kg of factor 8 raises the activity level 2%
Recombinant factor 9 dosing:1.5 unit/kg of r-factor 9 raises the acvity level 1%Beware anaphylaxis in F9 replacement
FUNDAMENTAL PRINCIPLE:when in doubt, treat!!!!
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
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 > 2 standard deviations below normal Determining reticulocyte count and MCV are first steps in determining etiology
Almost 1/3 of the world population is anemic!
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)
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
What's Wrong ?
What's Wrong ?
Sickle cell emergencies
Hgb S gene carried by 8% of African Americans1:625 have disease
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
38Sickle Cell Disease (SCD)General principles : Gentle hydrationAnti-inflammatory medications and narcotics .STAT CBC w Reticulocyte count , Type and cross.Supplemental oxygen ( controversial ) .
STROKE Can be ischemic (younger) or hemorrhagic (older ) .
Administer oxygen and IV fluids .CBC w retic, Type and cross .CT Scan brain .Alert Blood bank STAT - exchange transfusion .
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
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:
Temperature 38.5C>2 percent decrease in SpO2(O2saturation) from a documented steady-state value on room air (FiO2= 0.21)PaO2