Aggressive natural killer cell leukemia with skin manifestation associated with hemophagocytic lymphohistiocytosis: A case report

Abstract

Background: Aggressive natural killer cell leukemia (ANKL) is a rare natural killer cell neoplasm characterized by systemic infiltration of Epstein-Barr virus and rapidly progressive clinical course. ANKL can be accompanied with hemophagocytic lymphohistiocytosis (HLH). Here, we report a case of ANKL with rare skin lesions as an earlier manifestation, accompanied with HLH, and review the literature in terms of etiology, clinical manifestation, diagnosis and treatment.

Case summary: A 30-year-old woman from Northwest China presented with the clinical characteristics of jaundice, fever, erythema, splenomegaly, progressive hemocytopenia, liver failure, quantities of abnormal cells in bone marrow, and associated HLH. The immunophenotypes of abnormal cells were positive for CD2, cCD3, CD7, CD56, CD38 and negative for sCD3, CD8 and CD117. The diagnosis of ANKL complicated with HLH was confirmed. Following the initial diagnosis and supplementary treatment, the patient received chemotherapy with VDLP regimen (vincristine, daunorubicin, L-asparaginase and prednisone). However, the patient had severe adverse reactions and complication such as severe hematochezia, neutropenia, and multiple organ dysfunction syndrome, and died a few days later.

Conclusion: This is the first reported case of ANKL with rare skin lesions as an earlier manifestation and associated with HLH.

Keywords: Aggressive natural killer cell leukemia; Case report; Diagnosis and treatment; Epstein–Barr virus; Hemophagocytic lymphohistiocytosis; Rare skin lesions.

Figure 1

Physical examination and positron emission tomography/computed tomography scan (PET/CT). A: The forehead, behind the ears, neck and chest had dense flaky erythema with scattered bleeding spots of different sizes; B: PET/CT showed that metabolism was obviously increased in the right nasal cavity, right maxillary sinus, right frontal sinus, partial ethmoid sinus, bilateral sphenoid sinus, right turbinate, nasopharynx, uterus, tail of pancreas, bilateral breast, multiple lymph nodes, liver and spleen, and bone marrow.

Figure 2

Hematoxylin–eosin staining. A: Bone marrow cell morphology test showed that cells with unknown classification and abnormality were easy to see, accounting for 94% (magnification: 4 × 10 and 10 × 10); B: Bone marrow biopsy test showed that hematopoietic tissue proliferation was heterogeneous, adipose tissue hyperplasia was decreased, granulocyte and erythrocytic proliferation was decreased, megakaryocytic hyperplasia (0–4/high-power field) was scattered (magnification: 10 × 10 and 10 × 40); C: Immunohistochemical of bone marrow biopsy showed that the atypical cells were positive for CD2, cCD3, CD7, CD20, CD34, CD68, CD56, and negative for sCD3 (magnification: 10 × 10; 10 × 40 and 10 × 10).