Neonatal leukaemia.

Neonatal leukaemia is defined as occurring within the first 28 days of life and most, if not all, cases are congenital. With the exception of Down syndrome-associated transient abnormal myelopoiesis, which is not considered here, neonatal leukaemias are rare. In two-thirds of patients the disease manifests as an acute myeloid leukaemia, frequently with monocytic/monoblastic characteristics. Most other cases are acute lymphoblastic leukaemia, particularly B lineage, but some are mixed phenotype or blastic plasmacytoid dendritic cell neoplasms. The most frequently observed cytogenetic/molecular abnormality is t(4;11)(q21.3;q23.3)/KMT2A-AFF1 followed by t(1;22)(p13.3;q13.1)/RBM15-MKL1 and t(8;16)(p11.2;p13.3)/KAT6A-CREBBP. Common clinical features include prominent hepatosplenomegaly and a high incidence of skin involvement, sometimes in the absence of bone marrow disease. A distinctive feature is the occurrence of spontaneous remission in some cases, particularly in association with t(8;16). In this review, we summarise current knowledge of the clinical, cytogenetic and molecular features of neonatal leukaemia and discuss clinical management of these cases.

Congenital skin lesions caused by intrauterine infection with coxsackievirus B3.

BACKGROUND: Serious neonatal coxsackievirus infections transplacentally acquired in late pregnancy involve primarily the central nervous system, heart, liver and rarely the skin. PATIENTS AND METHODS: A boy born with a disseminated papulovesicular, nodular, bullous and necrotic ulcerated rash at 39 weeks gestational age developed pneumonia, carditis and hepatitis during the first days after birth. Molecular biological and serological methods were used for virological diagnosis. RESULTS: Coxsackievirus B3 (CVB3) was found in throat swabs and/or feces of the neonate and his mother. In addition, there was serological evidence of intrauterine infection. CONCLUSION: Intrauterine transmission of CVB3 during late pregnancy may lead to varicella-like congenital skin lesions.

Blueberry muffin rash as a presentation of alveolar cell rhabdomyosarcoma in a neonate.

Soft tissue sarcomas of childhood continue to present problems with pathologic diagnosis, staging and treatment. Rhabdomyosarcoma, the most common soft tissue sarcoma, represents 4-8% of all malignant solid tumours in children. We report a case of congenital alveolar rhabdomyosarcoma who presented with "blueberry muffin"-like rash. A full-term female infant was noted at birth to have multiple skin lesions resembling blueberry muffin rash and an abdominal mass in the left iliac fossa, which appeared to be fixed to the posterior abdominal wall. There was no enlargement of liver and spleen, but her para-aortic lymph nodes were enlarged. Biopsy from the mass confirmed the diagnosis of alveolar cell rhabdomyosarcoma. Molecular investigation for the t (2:13) translocation was negative. The infant received chemotherapy but died within 1 mo of diagnosis.

[Congenital Langerhans-cell histiocytosis presenting as a varicella infection]. / Kongenitale Langerhans-Zellen-Histiozytose unter dem klinischen Bild einer Varizelleninfektion.

UNLABELLED: We report on a preterm infant (33rd gestational week) with a varicella-like congenital rash, which initially appeared to respond to therapy with acyclovir. At the age of 3 weeks, lesions were in different stages of evolution and still resembled a varicella zoster virus (VZV) infection. However, since proof of VZV infection was lacking and new lesions erupted at the age of 4 weeks, a skin biopsy was performed which revealed a diagnosis of Langerhans cells histiocytosis. Therapy with prednisone resulted in prompt healing of the lesions. DISCUSSION: Congenital Langerhans cell histiocytosis is rare and symptoms may vary substantially from case to case. Like in our observation it may be confused with congenital varicella. In case of congenital skin lesions of uncertain etiology a skin biopsy should be performed.