Angioimmunoblastic T-cell Lymphoma Presenting as Purpura Fulminans
Purpura fulminans is a nonspecific hematologic emergency with high initial mortality, representing a thrombotic occlusion of blood vessels leading to skin necrosis and disseminated intravascular coagulation, and often reported in the setting of sepsis. We report a case of nonfatal purpura fulminans in the context of angioimmunoblastic T-cell lymphoma (AITL).
Practice Points
- Angioimmunoblastic T-cell lymphoma (AITL) is a primary nodal lymphoma with occasional nonspecific cutaneous involvement that may be morbilliform, maculopapular, erythrodermic, or rarely purpuric.
- To arrive at the correct diagnosis of AITL, a nodal biopsy with immunochemistry is necessary.
- CD10 positivity is a good objective criterion for the diagnosis of AITL, and Epstein-Barr virus–positive lymphocytes are nearly always present.
The development of purpura fulminans and disseminated intravascular coagulation in a patient with AITL is rare. Although the exact mechanism for the thrombus formation in the skin has not been elucidated, purpura fulminans typically develops secondary to a severe infection. The exact incidence of purpura fulminans in the setting of AITL is unknown, but purpura as a cutaneous eruption has been associated as a clinical finding in AITL.6 Although our case may be a rare presentation of AITL, a prompt and accurate diagnosis can drastically change the prognosis of this aggressive disease.
