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Case Reports
. 2023 Feb 16;11(5):1086-1093.
doi: 10.12998/wjcc.v11.i5.1086.

Angioimmunoblastic T-cell lymphoma induced hemophagocytic lymphohistiocytosis and disseminated intravascular coagulopathy: A case report

Mei Jiang  1 , Jing-Hua Wan  1 , Yi Tu  2 , Yan Shen  3 , Fan-Cong Kong  4 , Zhang-Lin Zhang  5
Affiliations
Case Reports

Angioimmunoblastic T-cell lymphoma induced hemophagocytic lymphohistiocytosis and disseminated intravascular coagulopathy: A case report

Mei Jiang et al. World J Clin Cases. .

Abstract

Background: Angioimmunoblastic T-cell lymphoma (AITL) is a subtype of peripheral T-cell lymphoma, with heterogenous clinical manifestations and poor prognosis. Here, we report a case of AITL induced hemophagocytic lymphohistiocytosis (HLH) and disseminated intravascular coagulopathy (DIC).

Case summary: An 83-year-old man presented with fever and purpura of both lower limbs for one month. Groin lymph node puncture and flow cytometry indicated a diagnosis of AITL. Bone marrow examination and other laboratory related indexes indicated DIC and HLH. The patient rapidly succumbed to gastrointestinal bleeding and septic shock.

Conclusion: This is the first reported case of AITL induced HLH and DIC. AITL is more aggressive in older adults. In addition to male gender, mediastinal lymphadenopathy, anaemia, and sustained high level of neutrophil-to-lymphocyte ratio may indicate a greater risk of death. Early diagnosis, early detection of severe complications, and prompt and effective treatment are vital.

Keywords: Angioimmunoblastic T-cell lymphoma; Case report; Disseminated intravascular coagulopathy; Hemophagocytic lymphohistiocytosis; Prognostic factors.

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Conflict of interest statement

Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.

Figures

Figure 1
Figure 1
Examinations. A: Purpura was observed on both lower limbs of the patient; B: Groin lymph node puncture specimen showed that the normal structure of lymph nodes disappeared and heterogeneous infiltration of small to medium-sized lymphoma cells, with proliferation of eosinophils (hematoxylin and eosin staining, × 40); C-E: Flow cytometry. Neoplastic T cells are shown in red and benign T cells in blue (analysis was gating on lymphocytes). The neoplastic T cells were positive for CD3, CD4, CD10, and PD1, but negative for CD7 and CD8; F: Bone marrow examination showed hemophagocytosis; G: Capillary electrophoresis revealed monoclonal IgG kappa.
Figure 2
Figure 2
Positron emission tomography. A-F: Positron emission tomography showed generalized lymphadenopathy, enhanced activity in the posterior pharyngeal wall (A), bilateral neck (B), hilum of the lung and mediastinum (C), pelvic wall (D), mesenteric lymph nodes (E), and groin (F).
See this image and copyright information in PMC

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