Abstract
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.
Highlights
Disseminated intravascular coagulation (DIC) is common in critically ill patients and is usually secondary to sepsis
We present a case of DIC initially managed supportively as being secondary to sepsis, with workup leaving HLH secondary to anaplastic large cell lymphoma (ALCL)
Primary systemic ALCL is a type of peripheral T-cell lymphoma which comprises about 2 percent of all non-Hodgkin’s lymphomas. ese are generally aggressive and four clinical subtypes exist: anaplastic lymphoma kinase- (ALK-) positive ALCL, ALK-negative ALCL, breast implant-associated ALCL, and primary cutaneous ALCL [2]
Summary
Disseminated intravascular coagulation (DIC) is common in critically ill patients and is usually secondary to sepsis It can potentially confound a diagnosis of a fatal and uncommon disorder such as hemophagocytic lymphohistiocytosis (HLH). 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 At presentation, she was tachycardic, hypotensive, and hypoxic. Laboratory workup showed hemoglobin 13 g/dL, white blood cell count 2.61 × 109/L, platelets 92 × 109/L, alkaline phosphatase 212 IU/L, aspartate aminotransferase 166 IU/L, alanine aminotransferase 61 IU/L, and a normal coagulation profile. A bone marrow biopsy showed many histiocytes and Laboratory parameter (units) Hemoglobin (g/dL) White blood cells (109/L) Platelets (109/L) PT (seconds) PTT (seconds) Lactate dehydrogenase (IU/L) Fibrinogen (mg/dL) Haptoglobin (mg/dL) Ferritin (ng/mL) Total bilirubin (mg/dL) Direct bilirubin (mg/dL) Alkaline phosphatase (IU/L) Aspartate aminotransferase (IU/L) Alanine aminotransferase (IU/L)
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