ALK-Negative Anaplastic Large Cell Lymphoma Presenting as Disseminated Intravascular Coagulation and Hemophagocytic Lymphohistiocytosis: A Potentially Fatal Presentation

• Published in: Case reports in hematology Vol. 2018; no. 2018; pp. 1 - 3
• Main Authors: Atallah, Jean Paul, Rehan, Maryam, Saqib, Amina, Ibrahim, Uroosa
• Format: Journal Article
• Language: English
• Published: Cairo, Egypt Hindawi Publishing Corporation 01-01-2018; Hindawi; John Wiley & Sons, Inc; Hindawi Limited
• Subjects: Blood; Bone marrow; Case Report; Case reports; Chemotherapy; Dehydrogenases; Development and progression; Disseminated intravascular coagulation; Family medical history; Ferritin; Fever; Haptoglobin; Laboratories; Lymphatic diseases; Lymphatic system; Lymphoma; Lymphomas; Medical prognosis; Mortality; Patients; Phosphatase; Sepsis; Tomography; Vincristine
• ISSN: 2090-6560; 2090-6579
• DOI: 10.1155/2018/3465351

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder that can be familial in etiology or a result of infections, malignancy, and autoimmune or inflammatory disorders. Disseminated intravascular coagulation (DIC) is common in patients admitted to intensive care units and can confound and delay the diagnosis of HLH. We present a case of a 69-year-old female who presented with dyspnea and malaise. Her condition declined rapidly with laboratory parameters consistent with DIC. In addition, she had a ferritin of 32,522 ng/mL, low haptoglobin, and elevated LDH, and bone marrow biopsy showed hemophagocytic lymphohistiocytes. She was started on HLH-directed therapy, and later, a diagnosis of ALK-negative anaplastic large cell lymphoma was made on an excisional inguinal lymph node biopsy specimen. Our case emphasizes the importance of prompt recognition, diagnosis, and treatment of HLH while workup for a primary disorder is still being pursued.