[A new case of congenital leukemia with leukemia cutis]. / Un nuevo caso de leucemia congénita con leucemia cutis.

Congenital leukaemia is a condition occurring very rarely. In a recent review in 1993, 175 cases are reported, 25-30% of them being well documented as leukaemia cutis. We reported a new case of congenital leukaemia diagnosed as an acute non lymphoblastic leukaemia M4 (FAB) and diagnosed at birth. It involves a newborn female at 42 weeks of gestational age. The most relevant clinical features were hepatomegaly and cutaneous petechial lesions along with a generalized distribution of nodules. From the blood peripheral count, leukocytosis is observed (177 x 10(9)/L) with 48% blasts of myeloid immunophenotype. The coagulation studies were consistent with a disseminated intravascular coagulation syndrome. A biopsy carried out on a cutaneous nodule, revealed diffuse dermoepidermic infiltration by immature cells of myeloid lineage, with cellularity and count similar to that of bone marrow and peripheral blood. The karyotype in the peripheral blood was normal. Infectious and immune causes were excluded as well as constitutional illnesses associated with unstable haematopoiesis. The family rejected treatment with chemotherapy and the baby died on day 53 of life due to progressive leukocytosis and concurrent infection. Our case, like 80% of the cases reported, is of myeloid origin and confirms the fatal evolution of untreated congenital leukaemia.

Immuno- and cytochemical characterization of congenital leukemia: a case report.

Congenital leukemia (CL) is a rare disorder, which usually presents as a myelocytic leukemia based on morphological features. Very few reports include data on cyto- or immunochemical parameters. A case of CL with detailed description of immunochemical, cytochemical features, and colony formation properties of the progenitor cells is reported. This leukemia was classified as an M4 in FAB classification based on the morphological features and special stains. Two different cell populations were identified by flow cytometry. One consisted of small cells expressing early T- and early B-cell associating antigens as well as early myeloid-associated antigens, but not CD10 (CALLA antigen), which is normally present in cord blood lymphocytes. The other was a population of large cells expressing myeloid-associated antigens and a pan-T-antigen. The growth pattern of hematopoietic progenitors in the patient was compatible with both acute myeloid and acute lymphatic leukemia as well as erythroleukemia.

Congenital leukemia: report of two cases.

Congenital leukemia is a rare disease in which a leukemic process is present at birth or immediately thereafter. The majority of cases presented in the literature were reported prior to the availability of contemporary immunophenotyping methods, and lineage assignment was often made on the basis of morphology alone. Congenital leukemias may be of various lineages, although, historically, monocytic and myelomonocytic congenital leukemias appear to be the most prevalent. We present two cases of congenital leukemia with detailed immunophenotypic and cytochemical characterization. One case is of the lymphoid lineage, and the second is of myelomonocytic lineage. Neither patient displayed trisomy 21.

Spontaneous remission of congenital leukemia.

Most patients with congenital leukemia do not survive past infancy despite aggressive chemotherapy. We describe three patients with congenital leukemia who have undergone prolonged periods of spontaneous remission. Our experience suggests that some patients with congenital leukemia may benefit from initial conservative management without chemotherapy. We summarize the clinical presentations of these patients and review the literature.