FEATURES OF DIAGNOSIS AND ADVERSE COURSE OF NEONATAL JUVENILE XANTHOGRANULOMA: CASE REPORT.

The work describes a case of rare neonatal systemic juvenile xanthogranuloma with an initial damage of the scalp, limbs, back and abdomen, multiple damages of the parenchyma of both lungs, spleen and liver with the development of a severe form of congenital cholestatic hepatitis. The diagnosis was established on the basis of histopathological and immunohistochemical examination of the skin nodules. The child on the background of therapy under the Langerhans cell histiocytosis III program achieved a partial response, which was manifested by a reduction of granulomatous formations on the skin, elimination of liver failure, but retained hepatosplenomegaly, specific lesions of the lung parenchyma, liver, and left kidney. Against the background of cytostatic therapy, the patient developed secondary pancytopenia, perianal ulcerative-necrotic dermatitis with lesions on buttocks, stomatitis, protein-energy deficiency, acute liver failure. coagulopathy, disseminated intravascular coagulation syndrome, acute renal failure, respiratory failure of III degree, cardiovascular insufficiency of III degree, pulmonary edema, cerebral edema, cerebral coma of II-III degree, enterocolitis, intestinal paresis. Despite multicomponent intensive care, the child's condition progressively deteriorated, and the patient died. The aspects of differential diagnosis of neonatal systemic juvenile xanthogranuloma are discussed.

Woman with Turner syndrome and her child with acute leukemia (a case report).

Turner syndrome (TS) is a chromosomal condition that affects development in females. The case of TS in the mother whose child was diagnosed with acute leukemia at the age of 1.5 years is presented. FANCI gene in child was detected among 94 genes associated- with hematologic malignancies. Acute lymphoblastic leukemia, common-B ІІ, L1, associated with t(12;21)(p13;q22), TEL/AML1 (ETV6/RUNX1) in a child was detected during a prophylactic examination. During the treatment of the baby, the mother had a second pregnancy, which ended in miscarriage at 8 weeks. Upon cytogenetic examination in the mother TS was revealed - mos45,Ð¥[23]/46, ХХ[7], and the father's karyotype was without abnormalities (46, ХУ). After chemotherapy, the child is in clinical-hematological remission. It could be suggested that chromosomal abnormalities in mother with TS may cause the chromosomal instability and hematological malignancy in offspring.

Non-Hodgkin's lymphoma of nasopharynx diagnosed during pregnancy (a case from practice).

The article presents the case of 32 years old woman with non-Hodgkin's lymphoma of the nasopharynx who received the anticancer treatment at 21-26 weeks of pregnancy (20 courses of radiation in dose of 40 Gy on the right half of the nasopharynx). The pregnancy was performed by cesarean section at the term of 32 weeks, and a healthy girl was born. Timely diagnosis and correct treatment of non-Hodgkin's lymphoma give the woman a chance to have a healthy child.

Peculiarities of menstrual function in mothers of children with acute lymphoblastic leukemia.

PURPOSE: To analyse the peculiarities of menstrual function in a group of mothers having children with acute lymphoblastic leukemia (ALL). PATIENTS AND METHODS: The case-control method was used, taking into consideration the age at first menstruation, cycle regularity, duration of menstruation and discharge quantity. The case group included 160 mothers with children suffering from ALL and the control group included 160 mothers having healthy children of the same age and sex. RESULTS: Alterations of the menstrual function in mothers of patients with ALL occur with statistically higher rate as compared with mothers having healthy children. Mothers of pediatric patients with ALL showed later menarche (>or= 15 years of age), reduced (to 2 days) or prolonged (over 6 days) menses duration, as well as irregularity of the menstrual cycle combined with prolonged menses duration. CONCLUSION: Menstrual disorders in mothers of child ren with ALL occur with reliably higher frequency as compared with women having healthy children. A common origin of both events (predisposition to malignancies and menstrual disorders) is suggested pointing to inherited genomic instability.

[Clinical and genealogical characteristics in families with Nijmegen breakage syndrome].

Nijmegen breakage syndrome (NBS) is a rare autosomal recessive disorder characterized by microcephaly, immunodeficiency and high predisposition for malignancies, particularly B-lymphoma. Clinical and genealogical analysis has been conducted in 7 families with NBS. Eight children with NBS (5 boys and 3 girls) were observed at the age from 7 months to 11 years. All the children were homozygous carriers for mutation 657del5. Oncohematological complications developed in 5 cases (4 cases of lymphoma and one case of lymphohystiocytosis) at the age of 6-12 years. NBS in probands is often accompanied with birth defects, especially with kidney pathologies. Considerable reproductive losts in the families with NBS were noted mainly among males who died at the age less than one year (4-6 events in the families). The cases of digestive system cancers (stomach, rectum, duodenum) were revieled in the family-trees. Consanguineous couple was observed in 1 case (marriage between third cousins) and 2 children had developed NBS in this family. Genealogical analysis seems to be very informative to predict somatic and reproductive disturbances in NBS families.