A Challenging Case of Coagulopathy in the ICU

Abstract

SESSION TITLE: Critical Care 2 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Disseminated Intravascular Coagulation (DIC) is common in critically ill patients and is usually secondary to sepsis. It can potentially confound a diagnosis of a potentially fatal and uncommon disorder such as hemophagocytic lymphohistiocytosis (HLH)1. Both entities are clinicopathologic diagnoses with shared coagulation system abnormalities. We present a case of DIC initially managed supportively as being secondary to sepsis, with workup leaving secondary HLH. CASE PRESENTATION: A 69 year old female with no known past medical history was brought to the hospital by ambulance for worsening shortness of breath and lethargy. The patient was recently treated for pneumonia with oral azithromycin. At presentation, the patient was tachycardiac, hypotensive and hypoxic with a hemoglobin of 13 g/dL, WBC count 2.61 x 109/L, platelets 92 x 109/L, alkaline phosphatase 212 IU/L, AST 166 IU/L, ALT 61 IU/L and a normal coagulation profile. In a week, her pancytopenia worsened PTT was 99 seconds, PT 13.7 seconds and fibrinogen 84 mg/dL. A CT scan of the abdomen showed necrotic right inguinal adenopathy the biopsy of which was non-diagnostic. Infectious disease work up was negative. Further studies showed a ferritin of 32522 ng/mL, haptoglobin of <20 mg/dL, LDH of 982 IU/L and low natural killer cell activity. A bone marrow biopsy showed significant hemophagocytic macrophages and a diagnosis of concomitant DIC and hemophagocytic lymphohistiocytosis was made. Dexamethasone 10 mg/m2 was started and she received two doses of etoposide 150 mg/m2 resulting in a decrease in ferritin to 19947 ng/mL and normalization of coagulation parameters. A repeat excisional inguinal lymph node biopsy was consistent with anaplastic large cell lymphoma. DISCUSSION: Hemophagocytic lymphohistiocytosis results from uncontrolled activation of cytotoxic T cells resulting in a cytokine storm. It can be familial or secondary to an underlying disorder and has a mortality rate of up to 70% from multiorgan failure. Our patient was started on HLH directed therapy based on clinical judgement and later fulfilled five diagnostic criteria CONCLUSIONS: Hemophagocytic lymphohistiocytosis and co-existent DIC is uncommon and can delay a diagnosis of HLH. Prompt recognition and treatment of the underlying disorder is crucial to improving outcomes. Reference #1: Coagulation Disorders and Bleedings in Critically Ill Patients With Hemophagocytic Lymphohistiocytosis.Valade S, Azoulay E, Galicier L, Boutboul D, Zafrani L, Stepanian A, Canet E, Lemiale V. 2015 Oct;94(40):e1692. DISCLOSURE: The following authors have nothing to disclose: Amina Saqib, uroosa Ibrahim, Gwen Garcia, Rabih Maroun, Jean Atallah No Product/Research Disclosure Information