Hematology

1. Hematology/Oncology Emergencies Stuart H. Gold July 25, 2010

2. Case presentation 12 yo AA male with a PMH of Sickle cell disease and severe hemophilia A presents with a 2 week history of fevers, LE pain, cough, epistaxis, and swelling and plethora of the upper chest and face Initial PE with significant for redness and swelling of face, cervical adenopathy and massive HSM, temp of 105, BP 63/22, P180, with swollen calf with cool toes

3. Case presentation Initial laboratory evaluation with WBC of 530k, hgb 3.5, plt 20k, PTT92(ULN 34), PT21 (ULN 14) LDH 10,000, Uric acid 12, PO4 8 CXR with large mediastinal mass

4. Emergencies Infection Anemia Bleeding Tumor Lysis Spinal cord compression Superior vena cava syndrome Hyperleukocytosis Sickle Cell emergencies Hemophilia emergencies

5. Infection Fever in the immunocompromised host is an emergency Chemotherapy Steroids Asplenia Immunodeficiency

6. Granulocytopenia Most important risk factor 7-10 days post chemo Especially when ANC<500 Bacterial, fungal, viral, mycobacterial Mucositis!! AML therapy

7. Treatment Stat blood counts, blood cultures (all lumens) Stat antibiotic Oncology (Cefepime, Vanc sometimes) Asplenia (Ceftriaxone) Patient does not sit around ER Local ER if distant (>1 hour)

8. Organisms Oncology Gram negatives - psuedomonas coverage AML patient/HD cytarbine - strep viridans Central line organism No bug is a contaminant Asplenia Encapsulated organisms

9. Persistent fevers 5 days of fever and neutropenia Think invasive fungal disease Amphotericin 0.5 mg/kg/d, ambisome 3- 5mg/kg, micafungin +/- vori Think of scanning Sinus, Lung, Liver/Spleen ?Anaerobic coverage

10. Pulmonary Infections Respiratory distress/Radiographic changes Think bronchoscopy or open lung bx Pneumocystis Prophylaxis

11. Varicella Exposures VZIG Vaccine Household Disease Hospitalize and treat IV acyclovir

12. Anemia Hypoproductive anemia usually not emergent, even if hgb 2 Irradiated, CMV safe Slow transfusion Beware of hyperviscosity Hemorrhagic/hemolytic anemia - more emergent

13. Anemia Blood volume In ml = 80 x body weight(kg) {85 for neonate} Amt of packed red cells to raise hematocrit (Blood vol X desired hct) � (blood volume X current hct)/hct of pRBC�s transfused

14. Hemorrhage Thrombocytopenia Usually not till <10,000 CMV safe, Irradiated if possible Avoid ASA, nonsteroidals Estrogens for excessive menses ??treat just numbers 6 units per square meter raise plts ~75K 1 apheresis pack = 6 units

15. Coagulation Factor Abn Hemophilia DIC Liver Disease Vitamin K deficiency

16. Hemophilia Replete appropriate factor Emergent Airway, Head, Paraspinal trauma, Compartment syndromes DDAVP mild FVIII pts Beware of inhibitor pt

17. Factor replacement FFP has everything 10cc/kg raises 10-20% CRYO VIII, XIII, Fibrinogen DDAVP Specific factor replacement Activated complexes, VIIa

18. Metabolic/Tumor Lysis Leukemia -esp ALL Lymphoma - esp Burkitt�s High tumor burden High LDH Fast growing tumors Start of therapy

19. Tumor Lysis Hyperuricemia Hyperphosphatemis Hyperkalemia Concommitant hypocalcemia

20. Treatment Allopurinol/Rasburicase Hydration - 2 times maintenance, without potassium Alkalinization � not any more Phosphate binders if needed, start early Dialysis if needed

21. Spinal Cord Compression Neuroblastoma Other spinal tumors Steroids Chemo vrs Radiation

22. Superior Vena Cava Syndrome Hodgkins and Non-Hodgkins Lymphoma Extrinsic compression of vena cava Edema and plethora of face, neck upper torso Respiratory embarrassment MAJOR anesthetic risk Diagnosis and treatment to start quick! Steroids, XRT, chemo

23. Hyperleukocytosis >100,000 - metablolic/stasis esp AML Lungs, brain, and kidneys Don�t dehydrate Don�t transfuse packed red cells Exchange transfusion Diagnose and treat quickly

24. Sickle Cell Infection Stroke Priapism Splenic Sequestration Respiratory distress/chest syndrome