Diane M. Komp

HLH‐94: A treatment protocol for hemophagocytic lymphohistiocytosis

In January 1995, the Hemophagocytic Lymphohistiocytosis (HLH) Study Group opened its first international treatment study dedicated to the hemophagocytic lymphohistiocytoses. The main intention of the study protocol is to offer affected children therapy with a wellestablished chemotherapeutic regimen (epipodophyllotoxin and corticosteroids) in combinationwith a newer approach, immunotherapy with cyclosporin A. Subsequent bone marrow transplantation (BMT) is recommended to all children with an available donor. The purpose of this communication is to describe this approach to management, that intends to prolong survival and increase the cure rate for children throughout the world with this highly lethal disease [1].

Infantile neuroblastoma presenting with cutaneous blanching nodules.

An 8-week-old girl was seen because of firm, blue cutaneous nodules. A biopsy showed neuroblastoma. When rubbed, the nodules blanched and were surrounded with a halo of erythema. She had Horners syndrome, hepatomegaly, bone marrow and shaft invasion, and a high thoracic mass with signs of spinal cord compression. Blanching cutaneous nodules are a unique finding in neuroblastoma and may be the first sign of this disease.