Cutaneous intravascular NK-cell lymphoma

Intravascular NK/T‐cell lymphoma: a report of five cases with cutaneous manifestation from China

“…Recently, Deetz et al and Metcalf et al reported that the vessels associated with intravascular anaplastic large cell lymphoma were actually D2‐40 positive, whereas those associated with intravascular NK/T‐cell lymphoma cases were D2‐40 negative . More importantly, all of the reported intravascular anaplastic large cell lymphoma cases were EBV‐negative, whereas nearly all of the reported intravascular NK/T‐cell lymphoma cases were EBV‐positive . According to these results, intravascular anaplastic large cell lymphoma actually appears to be intralymphatic anaplastic large cell lymphoma, and its growth pattern is very similar to the intravascular pattern observed with cutaneous metastasis …”

“…The relationship between intravascular NK/T‐cell lymphoma and aggressive NK‐cell leukemia will require clarification in the future. Bone marrow examination has seldom been performed in reported cases . A bone marrow examination was performed for two of our cases, but no bone marrow involvement was observed.…”

“…Given that one of our cases presented with positive CD30 staining, intravascular NK/T‐cell lymphoma should be distinguished from intravascular anaplastic large cell lymphoma; the latter is characterized by anaplastic cells with prominent polymorphisms, whereas the former features medium‐sized and rather uniform hyperchromatic cells . The neoplastic cells found in intravascular anaplastic large cell lymphomas usually occlude the vessels and form closely packed nodules, whereas the neoplastic cells found in intravascular NK/T‐cell lymphomas are usually scattered within the vessels . Recently, Deetz et al and Metcalf et al reported that the vessels associated with intravascular anaplastic large cell lymphoma were actually D2‐40 positive, whereas those associated with intravascular NK/T‐cell lymphoma cases were D2‐40 negative .…”

“…Died of disease after 22 days 3 4 Female/24 Subcutaneous nodules and ulceration on the legs and trunk; high fever Died of disease after 4 months 4 4 Male/33 Subcutaneous nodules on the face, neck and leg; high fever Alive after 1 year 5 17 Female/71 Patches and nodules on the trunk and extremities Alive after 5 months 6 21 Male/40 Papules on legs and trunks; splenomegaly Died of disease after 14 months 7 18 Female/68 Patches on the trunk and extremities; fever Died of disease after 2 months 8 18 Male/22 Patches on the trunk and legs; splenomegaly and fever Died of disease after 2 months 9 19 Female/42 Plaques on the legs; malaise, dizziness and chilly sensations Alive after 14 months 10 20 Female/84 Patches and nodules on the trunk and legs Alive after 4 months 11 22 Female/38 Plaque on chest and back; fever Died of disease after 13 months 12 23 Male marrow examination was performed for two of our cases, but no bone marrow involvement was observed. In our case 5, flow cytometry examination of the peripheral blood showed 71% of lymphocytes were positive for CD56, suggesting a leukemia distribution of the neoplastic cells.…”

“…Cerroni et al reported four cases with an NK or T‐cell immunophenotype and reviewed similar cases in the literature, concluding that intravascular NK/T‐cell lymphoma is a highly aggressive lymphoma that is usually associated with Epstein‐Barr virus (EBV) infection. Despite the rarity of intravascular NK/T‐cell lymphoma, it appears prone to development in Chinese patients . In this article, we describe our observations of 5 cases of intravascular NK/T‐cell lymphoma with cutaneous manifestation and review 12 previously reported similar cases involving Chinese patients.…”