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

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.

ALK-Positive Anaplastic Large Cell Lymphoma Associated With Hemophagocytic Lymphohistiocytosis

“…A literature review of ALCL-associated HLH in adults is presented in Table 2. Of the 14 cases found in the literature, eight patients improved and six patients died [6][7][8][9][10][11][12][13][14][15][16][17][18]. Seven patients were ALK-positive, five ALKnegative, and two ALK unknown.…”

Anaplastic Large Cell Lymphoma Presenting as Haemophagocytic Lymphohistiocytosis with Underlying Coxiella burnetii and Bartonella henselae Seropositivity

“…DISCUSSION HLH involves uncontrolled activation of macrophages, which engulf erythrocytes, leukocytes and platelets in the bone marrow. HLH can be familial or secondary to malignancy, infections or autoimmune disorders [1,2] . At least five out of the following are required for diagnosis: fever, splenomegaly, hypofibrinogenaemia, cytopenias, hypertriglyceridaemia, hyperferritinaemia, haemophagocytosis, low NK-cell activity and an elevated soluble CD25 level [1,2] .…”

“…HLH can be familial or secondary to malignancy, infections or autoimmune disorders [1,2] . At least five out of the following are required for diagnosis: fever, splenomegaly, hypofibrinogenaemia, cytopenias, hypertriglyceridaemia, hyperferritinaemia, haemophagocytosis, low NK-cell activity and an elevated soluble CD25 level [1,2] . Q fever resulting from CB infection is associated with a higher risk for both lymphomas and HLH.…”