Reacciones leucemoides en el recién nacido / Leukemoid reactions in the newborn

Presentamos el caso de una recién nacida prematura y de bajo peso al nacer que desarrolló cuentas leucocitarias elevadas hasta más de 100,000 x mmc, sin otras anormalidades hematológicas y que resolvió espontáneamente. A propósito del caso se revisó las ca usas de reacciones leucemoides en el período neonatal. En primer lugar la causa más conocida: síndrome míeloprollferatlvo transitorio, descrito en trisomía 21. En pacientes sin anomalías cromosómicas el uso de esteroides para inducir la maduración pulmonar , la corioamnlonitls y la prematurez extrema son exploradas como causas de RL. Luego revisamos el desarrollo de dlsplasia broncopulmonar y el síndrome de respuesta inflamatoria slstémlca en relación a RL...(AU)

In vitro cell growth stimulated by recombinant human cytokines can help to diagnose transient leukemia in neonates.

BACKGROUND/PURPOSE: In a previous study, we demonstrated that in vitro cell growth stimulated by human placental conditioned medium distinguished between transient leukemia (TL) and congenital acute myeloid leukemia (AML) in neonates. We then sought to determine whether the application can be expanded if in vitro cell growths are stimulated by recombinant human cytokines including granulocyte-macrophage colony-stimulating factor (rhGM-CSF), interleukin-3 (rhIL-3), stem cell factor (rhSCF) and thrombopoietin (rhTPO). METHODS: Eight neonates with features indistinguishable from AML were studied. Seven patients had Down syndrome and the eighth a normal phenotype. Bone marrow or peripheral blood mononuclear cells (MNC) were cultured in the presence of rhGM-CSF+rhIL-3+rhSCF or of rhTPO alone. After incubation, granulocyte-macrophage colony-forming units (CFU-GM)-derived colonies and clusters were scored on an inverted microscope. Colony-forming units-megakaryocyte (CFU-MK)-derived colonies were counted with an in situ CD61 immunostained dish. Liquid suspension cultures of MNC were stimulated by rhGM-CSF and/or rhTPO. RESULTS: CFU-GM-derived colonies and clusters from bone marrow and peripheral blood MNC revealed normal patterns in seven patients. RhTPO-stimulated megakaryocyte colony formation was normal in one patient. Cytospin smears of liquid suspension cultures all showed good myeloid or megakaryocytic maturation consistent with TL rather than AML. One neonate died on the 2nd day of life, but in the seven remaining patients, blasts disappeared from the peripheral blood within 10 months. Among four patients followed long-term, one developed myelodysplastic syndrome at 21 months. This child was given tailored chemotherapy and had a disease-free survival>20 months. CONCLUSION: In vitro cell growth stimulated by recombinant human cytokines can help to diagnose TL in neonates.

An unusual pustular eruption in an infant with Down syndrome and a congenital leukemoid reaction.

Congenital leukemia and leukemoid reactions may be indistinguishable on clinical and histologic grounds and are highly associated with trisomy 21. This report characterizes a specific vesiculopustular skin eruption in an infant with Down syndrome and a congenital leukemoid reaction. On the first day of life an unusual vesiculopustular eruption developed, starting in areas of cutaneous trauma. A biopsy revealed immature myeloid cells in an epidermal spongiotic vesiculopustule and in a perivascular distribution, suggestive of leukemia cutis. As the peripheral blood smear normalized, the eruption cleared. Myelodysplasia subsequently developed and evolved into acute myelogenous leukemia. This is the first detailed report of a specific skin infiltrate caused by the immature cells of a leukemoid reaction. Skin infiltration by immature myeloid cells during a congenital leukemoid reaction may portend an aggressive course of the myeloproliferative disorder.

Karyotype evolution in a patient with Down syndrome and acute leukemia following a congenital leukemoid reaction.

We report the serial cytogenetic study of a patient with Down syndrome who experienced a congenital leukemoid reaction, underwent a spontaneous remission within four months, and subsequently developed acute myeloid leukemia at 16 months. A blood chromosome study to rule out Down syndrome performed at age 24 days, during the leukemoid reaction, revealed a 47,XX,+21 karyotype. The diagnosis of acute leukemia was made at 16 months, at which time a chromosome study, on bone marrow, was performed. This analysis revealed a clonal karyotype of 47,XX,+21,-22,+der(22)t(1;22)(q21;q13) in all but one cell studied. The single apparently nonclonal cell showed a karyotype of 49,XX,+12,-13,-19,+der(19)t(19;?)(q11;?)x2,+21,+22. A third chromosome study at 19 months indicated the original leukemic clone with t(1;22) (q21;q13) had been replaced by the clone represented by the single cell with 49 chromosomes seen in the previous chromosome study. This case of an infant with Down syndrome and acute leukemia illustrated rapid evolution and a transitory nature to clonal chromosome aberrations while retaining AML morphology and course.

Congenital leukemoid reaction followed by fatal leukemia. A case with Down's syndrome.

A serial clinical, hematologic, and cytogenetic study was done on a baby with Down's syndrome in whom a myeloid leukemoid reaction developed at birth that spontaneously regressed within a month only to relapse two years later to an acute undifferentiated stem cell leukemia. He died 1 1/2 months after onset. The unresolved controversy of the diagnosis of the congenital leukemia-like state is discussed. The importance of following up such patients with apparent remission of their congenital leukemia-like disorder is emphasized.