Biomedical subjects

Halina Bubała

Publications and source records attributed to Halina Bubała.

9 recordsLinked to original sources

PubMed2006

ALK-positive diffuse large B-cell lymphoma.

Anaplastic lymphoma kinase (ALK) positive diffuse large B-cell lymphoma (DLBCL) is a rare subtype of non-Hodgkins lymphoma. Five such cases have been described in children. We present a 9-year-old boy, in whom diagnosis of DLBCL has been established in addition to congenital multiple enchondromatosis. Immunohistopathological evaluation of tumor biopsy established the final diagnosis of ALK + DLBCL. The clathrin gene (CLTC)-ALK fusion underlying aberrant expression of ALK in the present case was demonstrated by interphase fluorescence in situ hybridization (FISH) using break-apart rearrangement probes for ALK and CLTC. The disease in this patient was highly resistant to applied chemotherapy regimens and to radiotherapy. Analysis of the disease course in our patient and review of other cases reported previously show that ALK + DLBCL can be an aggressive malignancy that can be cured with conventional chemotherapy protocols only at stage of localized disease.

Anaplastic Lymphoma Kinase↗

PubMed2006

Carrier frequency of mutation 657del5 in the NBS1 gene in a population of Polish pediatric patients with sporadic lymphoid malignancies.

Nijmegen breakage syndrome (NBS) is a human autosomal recessive disease characterized by genomic instability and enhanced cancer predisposition, in particular to lymphoma and leukemia. Recently, significantly higher frequencies of heterozygous carriers of the Slavic founder NBS1 mutation, 657del5, were found in Russian children with sporadic lymphoid malignancies, and in Polish adults with non-Hodgkin lymphoma (NHL). In addition, the substitution 643C>T (R215W) has also been found in excess among children with acute lymphoblastic leukemia (ALL). In an attempt to asses the contribution of both mutations to the development of sporadic lymphoid malignancies, we analyzed DNA samples from a large group of Polish pediatric patients. The NBS1 mutation 657del5 on one allele was found in 3 of 270 patients with ALL and 2 of 212 children and adolescents with NHL; no carrier was found among 63 patients with Hodgkin lymphoma (HL). No carriers of the variant R215W were detected in any studied group. The relative frequency of the 657del5 mutation was calculated from a total of 6,984 controls matched by place of patient residence, of whom 42 were found to be carriers (frequency = 0.006). In the analyzed population with malignancies, an increased odds ratio for the occurrence of mutation 657del5 was found in comparison with the control Polish population (OR range 1.48-1.85, 95% confidence interval 1.18-2.65). This finding indicates that the frequency of the mutation carriers was indeed increased in patients with ALL and NHL (p < 0.05). Nonetheless, NBS1 gene heterozygosity is not a major risk factor for lymphoid malignancies in childhood and adolescence.

Adolescent↗

PubMed2005

Decreased numbers of CD4+ T lymphocytes in peripheral blood after treatment of childhood acute lymphoblastic leukemia.

Treatment-related immunosuppression in patients with acute lymphoblastic leukemia (ALL) is associated with increased susceptibility to infectious diseases, also after the treatment. The aim of the present study was the detailed evaluation of T lymphocyte subsets in peripheral blood in children after treatment of ALL. All children were treated according to the BFM 90 protocol. The patients were divided into 5 groups of 30 children in each, depending on the time from cessation of the ALL treatment. A control group consisted of 30 healthy children subjected to elective "1-day" surgery. The children's age ranged from 6 to 18 years. The examinations were performed in FACScan flow cytometer with the use of wide set of monoclonal antibodies: CD3, CD4, CD8, TCRalphabeta, TCRgammadelta, CD19, CD25, CD45RA, CD45RO, CD69, HLA-DR, CD16 and CD56, which particularly allowed detailed analysis of T lymphocytes. The results showed that most parameters in children 1 year after ALL treatment completion were similar to healthy children. However, we observed persistently low CD4+ T cell numbers, both CD45RA+ as well as CD45RO+ subsets as compared to the control group. This might reflect decreased regenerative potential of immunological system in children 1 year after ALL treatment.

Adolescent↗

PubMed2004

[Sudden blindness of 11-year old boy in the course of acute lymphoblastic leukemia].

PURPOSE: The aim of this study is to present dramatic ocular manifestation of acute lymphoblastic leukemia. MATERIAL AND METHODS: The 11 years old boy was hospitalized due to acute leukemia at the Department and Clinic of Pediatric Hematology, and was referred to ophthalmic examination because of sudden blindness of the right eye. General ophthalmic examination and electrophysiological tests were done. RESULTS: No light perception of right eye, and markedly reduced visual evoked responses were caused by infiltration with great edema in optic nerve and its surrounding of retina. In spite of the fact, that the infiltrations disappeared after general therapy, the vision did not recover at all. CONCLUSIONS: Even intensive treatment may be ineffective in blindness prevention in severe course of acute leukemia.

Blindness↗

PubMed2004

[Acute lymphoblastic leukemia in a 3-week-old neonate].

We present a case of 3-week-old infant, in whom we established the diagnosis of congenital acute lymphoblastic leukemia (ALL), characterized by the immunophenotype of the most immature B-cell precursors (pro-B-ALL) and chromosomal translocation t(4;11) associated with the rearrangement of MLL and AF4 genes. The clinical course of this leukemia deserves particular attention, because it was different from classical presentation: diagnosis--not immediately after birth--but at the age of 3 weeks, no signs of hemorrhagic diathesis, lack of nodular skin infiltrates, and no central nervous system involvement. Exchange blood transfusions performed during the remission induction treatment saved the child from life-threatening symptoms of leukostasis at the initial stage of leukemia. Despite successful remission induction in bone marrow, the infant relapsed during the fifth month of treatment. After subsequent chemotherapy course the child died owing to fatal infectious complications. Despite enormous progress in treatment of childhood ALL, this type of leukemia in infants below 6 months of age, and particularly in cases of congenital leukemia is still associated with a very poor prognosis.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2004

[Progress in the treatment of non-Hodgkin's lymphoma (NHL) in children. The report of Polish Pediatric Leukaemia/lymphoma Study Group (PPLLSG)].

Treatment results of non-Hodgkin lymphoma (NHL) in children has been shown in this study. From 1979 to 2003 children were registered with the diagnosis of NHL in oncology centers of Polish Pediatric Leukaemia/Lymphoma Study Group, a group of 397 patients with NHL B, 222 pts with NHL T and 54 pts with anaplastic large cell lymphoma (ALCL). The pts with NHL T have been treated according to BFM-90 protocol. The predominant primary site of disease was mediastinum (59.3%). Complete remission (CR) was achieved by 87%. EFS for all NHL T pts was 65% and 56% for pts with extensive tumours and 73% for pts with tumours < 10 cm. Patients with NHL B were treated according to the adopted LMB-89 protocol. The majority were Burkitts type and presented abdominal location (50%). 80% with disseminated disease. CR was achieved by 89% patients, but 94% with LDH < 500 IU/L and 73% with LDH > 500 IU. The median time of follow up was 53 months. EFS was 73% for all patients. The patients with ALCL were treated according to several protocols. Peripheral nodes were the most often primary location (40%), than mediastinum (24%) and abdomen (21%). EFS for all pts was 63%. Despite great progress in the therapy of NHL in children during 20 years of observation, the results are not satisfactory in disseminated stages. It is necessary to look for new prognostic markers which make it possible to improve classification of patients. Major surgery in advanced stages is not recommended since it delays chemotherapy and fails to improve overall survival. Early detection of neoplasm is one of the most important efforts to improve therapy success.

Abdominal Neoplasms↗

PubMed2004

[Leukapheresis in children with chronic myeloid leukemia and pulmonary leukostasis].

We present two patients with chronic myeloid leukemia and hyperleukocytosis above 350 x 10(9)/l causing the symptoms of pulmonary leukostasis. Therapeutic leukapheresis procedures with concomitant chemotherapy were initiated within 12 hours from the diagnosis. After the therapy commencement the general condition of the patients systematically improved and leukocytosis gradually decreased from the second day of treatment with disappearance of pulmonary leukostasis symptoms. Thanks to application of such treatment procedures during the cytoreductive period, severe complications related to leukostasis could be prevented.

Adolescent↗

PubMed2004

[Acute endophthalmitis of 15-year old boy in the course of acute lymphoblastic leukemia--part I].

PURPOSE: The aim of this study is to present dramatic ocular manifestation of acute lymphoblastic leukemia. MATERIAL AND METHODS: 15-year old boy was hospitalized due to acute leukemia at the Department and Clinic of Pediatric Hematology and was referred to ophthalmologist because of strong pain and decreased visual acuity of the left eye. General ophthalmic examination and electrophysiology were done. These procedures were repeated after 1, 2, 5 and 11 weeks and also after 6 and 9 months. RESULTS: Acute uveitis with cellular exudation to the vitreous, papilledema, swelling of the macula and green-yellowish infiltration which elevated the temporal part of the retina, were noticed. Swelling of the iris and miosis were observed. After one week of local anti-inflammatory treatment the eye became painless with little deep injection only. Simultaneously causal treatment of basic disease was continued. Control examinations revealed step by step improvement of ocular changes. After 6 months in the place of retinal infiltration choroidoretinal atrophy was seen. The functional deficits in visual field and electrophysiological examinations were found, too. One year later, the patient came again because of recurrence. Involvement of the central nervous system with signs of meningitis occurred. Visual acuity was normal and no infiltration of eyes was found, but there was papilledema in both eyes. CONCLUSIONS: In acute leukemia ocular manifestation may be highly expressed. The patients require local symptomatic and general causal treatment in cooperation of ophthalmologist with hematologist.

Acute Disease↗

PubMed2003

[Tumor lysis syndrome in the course of acute lymphoblastic leukemia as the consequence of prednisone monotherapy].

Authors present the case of 15-year-old girl with diagnosed pre T-acute lymphoblastic leukaemia with hyperleukocytosis--at admission 213 x 10(9)/l. At clinical evaluation a major peripheral and abdominal lymphadenopathy, mediastinal mass, hepatosplenomegaly were revealed. After administration of prednisone for two times--11.4 mg/m2/24 h--the features of tumour lysis syndrome rapidly increased. Hyperphosphatemia, hyperkalemia and hyperuricemia resulted in acute renal failure. Renal replacement therapy was introduced with simultaneous application of cytoreduction phase therapy and induction of remission according to New York protocol. Total number of performed hemodialysis sessions was 12. Obtained haematological remission in 8th week of treatment is still present. Renal function remains normal. Remission supportive treatment has been continued according to above-mentioned protocol.

Adolescent↗