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Biomedical subjects

M V Gresik

Publications and source records attributed to M V Gresik.

At least 19 recordsLinked to original sources
PubMed2001

Cytochemical staining and flow cytometry methods applied to the diagnosis of acute leukemia in the pediatric population: an assessment of relative usefulness.

BACKGROUND: Cytochemical staining has been used in the diagnosis of acute leukemia for more than 20 years. The general availability of flow cytometers and an extensive panel of antibody reagents useful for characterizing blood cell lineage question the usefulness of continuing routine use of the cytochemical staining for the diagnosis of acute leukemia. PATIENTS AND METHODS: Test results were evaluated in 122 (n = 122; 112 with acute lymphocytic leukemia and 10 with acute myeloid leukemia) patients selected from among 320 patients with acute leukemia at Texas Children's Hospital in 1997 and 1998. Results were selected for review if the clinical encounter represented the initial diagnostic work-up and if data were available from cytochemical staining and flow cytometry studies. RESULTS: Cell lineage classification derived from flow cytometry and cytochemical stains were in agreement in all cases. Definitive diagnoses were feasible using flow cytometry results alone in 120 of 122 patients (98.4%) as compared with only 99 of 122 patients (81.2%) when only cytochemical staining results were considered. In two patients with inconclusive flow cytometry results, cytochemical staining alone provided information sufficient for diagnosis. CONCLUSIONS: Results from this study indicate that with few exceptions, flow cytometry studies alone provide sufficient information for diagnosis and management of acute leukemia in children. Nevertheless, cytochemical staining should be available for those cases in which flow cytometry results fail to allow a definitive diagnosis. A modified testing protocol is recommended.

Acute Disease↗
PubMed2000

Prognostic factors in children and adolescents with acute myeloid leukemia (excluding children with Down syndrome and acute promyelocytic leukemia): univariate and recursive partitioning analysis of patients treated on Pediatric Oncology Group (POG) Study 8821.

The purpose of the paper was to define clinical or biological features associated with the risk for treatment failure for children with acute myeloid leukemia. Data from 560 children and adolescents with newly diagnosed acute myeloid leukemia who entered the Pediatric Oncology Group Study 8821 from June 1988 to March 1993 were analyzed by univariate and recursive partitioning methods. Children with Down syndrome or acute promyelocytic leukemia were excluded from the study. Factors examined included age, number of leukocytes, sex, FAB morphologic subtype, cytogenetic findings, and extramedullary disease at the time of diagnosis. The overall event-free survival (EFS) rate at 4 years was 32.7% (s.e. = 2.2%). Age > or =2 years, fewer than 50 x 10(9)/I leukocytes, and t(8;21) or inv(16), and normal chromosomes were associated with higher rates of EFS (P value = 0.003, 0.049, 0.0003, 0.031, respectively), whereas the M5 subtype of AML (P value = 0.0003) and chromosome abnormalities other than t(8;21) and inv(16) were associated with lower rates of EFS (P value = 0.0001). Recursive partitioning analysis defined three groups of patients with widely varied prognoses: female patients with t(8;21), inv(16), or a normal karyotype (n = 89) had the best prognosis (4-year EFS = 55.1%, s.e. = 5.7%); male patients with t(8;21), inv(16) or normal chromosomes (n = 106) had an intermediate prognosis (4-year EFS = 38.1%, s.e. = 5.3%); patients with chromosome abnormalities other than t(8;21) and inv(16) (n = 233) had the worst prognosis (4-year EFS = 27.0%, s.e. = 3.2%). One hundred and thirty-two patients (24%) could not be grouped because of missing cytogenetic data, mainly due to inadequate marrow samples. The results suggest that pediatric patients with acute myeloid leukemia can be categorized into three potential risk groups for prognosis and that differences in sex and chromosomal abnormalities are associated with differences in estimates of EFS. These results are tentative and must be confirmed by a large prospective clinical trial.

Adolescent↗
PubMed1999

Chromosomal abnormalities in 478 children with acute myeloid leukemia: clinical characteristics and treatment outcome in a cooperative pediatric oncology group study-POG 8821.

We determined the type and frequency of chromosomal aberrations in leukemic cells of 478 children diagnosed with acute myeloid leukemia and enrolled in the Pediatric Oncology Group study 8821. Of the 478 cases, 109 (22.8%) had normal karyotypes. Chromosomal abnormalities of 280 patients (58.6%) were classified into subgroups: 11q23 abnormalities (n = 88, 18.4%), t(8;21) (n = 56, 11.7%), t(15;17) (n = 55, 11.5%), inv(16)/t(16;16) (n = 28, 5.9%), trisomy 8 alone (n = 10, 2.1%), monosomy 7 (n = 9, 1.9%), non-Down-associated trisomy 21 alone (n = 7, 1.5%), and rare recurrent chromosomal translocations (n = 27, 5.6%). The remaining 89 patients (18.6%) had miscellaneous clonal abnormalities. Overall, 84.9% of the children achieved a complete remission; the 4-year event-free survival (EFS) estimate was 33.8% +/- 2.4%. Remission rates were significantly higher (96.4%, P =.011) for patients with t(8;21) and inv(16)/t(16;16) but significantly lower (74.5%, P =.022) for those with t(15;17). The 4-year survival rate for all patients was 43.5% +/- 2.4%; for those with an inv(16)/t(16;16), 75.0% +/- 8.6%; a normal karyotype, 53.8% +/- 4.9%; a t(8;21), 51.6% +/- 7.3%; a t(15;17), 39.8% +/- 6.9%; and an 11q23 abnormality, 32.9% +/- 5.1%. Four-year EFS estimates for patients with inv(16)/t(16;16) (58.2% +/- 10.9%, P =.007), t(8;21) (45.1% +/- 7.7%, P =.014), or normal karyotypes (43.1% +/- 5.0%, P =. 012) were higher than the 4-year EFS estimate for all patients, but EFS estimates for patients with t(15;17) (19.6% +/- 8.0%, P =.033) or 11q23 abnormalities (23.8% +/- 4.8%, P =.0013) were lower. EFS estimates did not differ significantly among 11q23 subgroups. Limited analysis suggested that patients with inv(16) can be salvaged better following relapse than those with t(8;21). Thus, patients with an inv(16)/t(16;16) may have high survival rates when treated with chemotherapy alone.

Acute Disease↗
PubMed1998

Role of staging bone marrow examination in children with Hodgkin disease.

PURPOSE: To determine the value of bone marrow trephine biopsy as part of the clinical staging for children presenting with Hodgkin disease. PATIENTS AND METHODS: A retrospective study of pre-treatment bone marrow examinations was undertaken to examine the value of bone marrow staging in children with Hodgkin disease. The records of 122 children, diagnosed with Hodgkin disease at Texas Children's Hospital between February 1960 and July 1996 were reviewed. Age, sex, complete blood counts (CBC), pathology, and clinical and pathological staging results were tabulated. RESULTS: Information was complete for analysis in 110 patients. Bone marrow trephine biopsies identified Hodgkin disease in 2/110 patients (1.8%). The patients with bone marrow disease had clinical stage IIIB disease prebiopsy. Positive bone marrow biopsy results did not effect a change in therapy, and the small number of positive cases do not allow any prediction as to prognosis. CONCLUSION: There is no role for bone marrow trephine examination in children with clinical stage I-IIIA Hodgkin disease.

Adolescent↗
PubMed1998

Detection of double minute chromosomes in a child with acute lymphoblastic leukemia.

A child with acute lymphoblastic leukemia (ALL) whose predominant leukemic clone demonstrated double minute chromosomes (dmin) is presented. The patient had no history of mutagen or carcinogen exposure and responded well to combination chemotherapy. Although dmin have been described in acute myelogenous leukemia and various solid tumors in adults, their presence in childhood neoplasms is less frequent and limited primarily to neurogenic tumors. This is the first documentation of dmin in childhood ALL, suggesting that there may be an unrecognized subgroup of ALL patients with gene amplification.

Child↗
PubMed1995

Unique de novo interstitial deletion of chromosome 17, del(17) (q23.2q24.3) in a female newborn with multiple congenital anomalies.

We describe a newborn with a novel interstitial deletion of the long arm of chromosome 17 [del (17) (q23.2q24.3)] who died on day of life 17 during a recurrent apneic episode. Her phenotype included severe growth retardation, multiple facial anomalies, maldeveloped oralpharyngeal structures, and digital and widespread skeletal anomalies. This patient's phenotype was compared to two other reported patients with deletion 17q with minor clinical overlap consistent with a unique deletion.

Abnormalities, Multiple↗
PubMed1994

Isolated bone relapse during hematologic remission in childhood acute lymphoblastic leukemia: report of a metatarsal relapse and review of the literature.

An 11-year-old boy with prior bone marrow and testicular relapses of his acute lymphoblastic leukemia (ALL) developed an isolated metatarsal bone relapse during complete hematologic remission 10 months after completion of chemotherapy. Although there was no radiographic or histologic evidence of additional occult leukemia, the polymerase chain reaction (PCR) technique detected a leukemic clone in both his bone marrow and metatarsal. A literature survey revealed only 10 reported cases of isolated bone relapse occurring during complete bone marrow remission in childhood ALL. Most of these patients had prior bone marrow or extramedullary relapses. The majority experienced subsequent relapses after their isolated bone recurrence. We report a case of isolated bone recurrence, review all previously reported cases, and suggest that PCR elucidation of clonal disease may provide a better understanding of these extremely rare extramedullary events.

Humans↗
PubMed1993

Intraoral presentation of anaplastic large-cell Ki-1 lymphoma in association with HIV infection.

Persons infected with human immunodeficiency virus have an increased risk for development of high-grade, non-Hodgkin's lymphomas. Anaplastic large-cell Ki-1 lymphoma is a recently described lymphoid neoplasm characterized by cellular pleomorphism, a sinusoidal growth pattern, and Ki-1 epitope reactivity. This type of lymphoma is often mistaken for metastatic carcinoma, melanoma, or malignant histiocytosis. Although persons with acquired immunodeficiency syndrome frequently have non-Hodgkin's lymphoma at extranodal sites, the oral cavity and mandible, in particular, are unusual locations. We report two cases of anaplastic large-cell Ki-1 lymphoma that occurred in persons with the human immunodeficiency virus and with initial presentation as soft tissue masses of the posterior mandible. Immunocytochemical studies were positive for Ki-1 (CD30) in both cases. In situ hybridization for Epstein-Barr virus-deoxyribonucleic acid was positive with tumor cells in both cases. Flow cytometry on paraffin, formalin-fixed tissue revealed tetraploidy and high proliferative fractions that are characteristic of high-grade lymphomas. Intraoral presentation of rapidly enlarging, soft tissue masses may represent a high-grade non-Hodgkin's lymphoma in persons with the human immunodeficiency virus. Although rare, anaplastic large-cell Ki-1 lymphoma should be considered and requires immunocytochemical study to eliminate the possibility of other malignant conditions associated with the acquired immunodeficiency syndrome.

Adult↗
PubMed1993

B19 parvovirus-induced anemia in a normal child. Initial bone marrow erythroid hyperplasia and response to intravenous immunoglobulin.

PURPOSE: Human B19 parvovirus infection may cause severe erythroid hypoplasia in patients with an underlying hemolytic anemia. We report a case of severe parvovirus-induced anemia with initial marrow erythroid hyperplasia in a child with no underlying hematologic disorder. CONCLUSIONS: The patient's rapid hemoglobin recovery after treatment with i.v. immunoglobulin further supports this form of therapy for children with parvovirus-induced anemia.

Anemia↗
PubMed1992

A familial lymphoproliferative disorder presenting with primary pulmonary manifestations.

A familial lymphoproliferative disorder presented in three male siblings with primary pulmonary involvement manifested as either lymphoid interstitial pneumonia or an angiodestructive polymorphous infiltrate morphologically resembling lymphomatoid granulomatosis. The polymorphous infiltrate consisted chiefly of mature T-cells with a few B-cells and plasma cells, and gene rearrangement studies failed to show clonality. Epstein-Barr virus, frequently associated with proliferative lesions in males in the X-linked lymphoproliferative syndrome, was not demonstrated in any of the pulmonary lesions. An HLA haplotype shared among the affected siblings was A1, B8, DR4. The unusual clinical presentation plus the lack of involvement by EBV in the pulmonary lesions suggests that this is a previously undescribed familial lymphoproliferative disorder.

Adolescent↗
PubMed1992

Expression of myeloid-associated and lymphoid-associated cell-surface antigens in acute myeloid leukemia of childhood: a Pediatric Oncology Group study.

PURPOSE: Although the expression of both myeloid- and lymphoid-associated cell-surface antigens in acute myeloid leukemia (AML) has been described, the clinical significance of such antigen expression remains unknown in the pediatric population. We sought to define an antibody panel for optimal diagnostic antigenic analysis and to test associations among antigen expression and a number of clinical features at presentation and prognosis in pediatric AML. PATIENTS AND METHODS: We reviewed the extensive immunophenotypic analysis performed at the time of diagnosis on 132 assessable patients registered on a single Pediatric Oncology Group AML protocol between 1984 and 1988. RESULTS: Eighty-eight percent of patients were identified by testing for expression of CD33 and CD13. Overall, 61% of patients expressed at least one lymphoid-associated antigen, most commonly CD4, CD7, or CD19. Expression of CD5, CD10, CD20, or CD22, commonly detected in T- or B-lineage pediatric acute lymphoid leukemia (ALL), was uncommon; coexpression of multiple lymphoid-associated antigens was also uncommon. Expression of the monocyte-associated antigen CD14 correlated with French-American-British (FAB) M4 or M5 morphology. Otherwise, no correlation between antigen expression and FAB classification was noted. None of the myeloid, lymphoid, natural-killer (NK), or progenitor-associated antigens were associated with significant differences in the likelihood of remission induction or event-free survival when expressor versus nonexpressor groups were compared. CONCLUSIONS: The distribution of cell-surface antigen expression in pediatric acute leukemia usually permitted the discrimination of AML from ALL by using a limited panel of antibodies. Although the expression of lymphoid-associated antigens was common, such expression did not seem to be associated with an adverse prognosis in pediatric AML.

Acute Disease↗
PubMed1992

Progressive transformation of germinal centers: comparison of 23 pediatric patients to the adult population.

Approximately 10% of enlarged lymph nodes showing reactive follicular hyperplasia (RFH) will contain one or more progressively transformed germinal centers (PTGC). Comparison of 23 patients 16 yr old and younger (the pediatric group) of age to the adult population (greater than 16 yr old) indicates that most of the patients in each category present with a solitary asymptomatic enlarged lymph node (63% each group), usually cervical, and, while there may be recurrent lymphadenopathy showing RFH with PTGC (RFH/PTGC), evolution to Hodgkin's disease (HD) or other lymphomas is rare. Cases of PTGC unassociated with HD comprise the largest group: 70% (16/23 patients) of pediatric group; 60% (52/87 patients) of adult group. Five pediatric patients (22%) had antecedent HD (four males with lymphocytic predominance-nodular, NL&H HD; one female with nodular sclerosis, NSHD); this group is too small for comparison with adults. One patient (4%) subsequently developed NL&H HD; one patient had concurrent PTGC in the lymphoid rim surrounding NL&H HD. The 23 pediatric patients ranged from 4 to 16 yr (median 11, mean 11.3) with a male predominance (18 M, 5 F) similar to the adult population. Three differences occur comparing PTGC patients without associated HD from the two age groups. The less than or equal to 16 age group has a higher recurrence rate of PTGC (50 versus 23%); two or more biopsies showing PTGC after the initial biopsy was common, 19 versus 0%; and, morphologically, the pediatric cases unassociated with HD more often contain epithelioid histiocytic clusters (44 versus 0%), which may rim the PTGC.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1990

Typhlitis. An 18-year experience and postmortem review.

A review of pediatric autopsy results at the Texas Children's Hospital, Baylor College of Medicine from 1970 through 1987 was conducted. Thirty-three cases of typhlitis were identified in patients with acute leukemia and two cases each in patients with lymphoblastic lymphoma and aplastic anemia. Patients ranged in age from 10 months to 17 years. Fifty-seven percent were male and 43% were female. All were myelosuppressed. A postmortem incidence rate of 24% was determined for patients with acute leukemia. Common symptoms included abdominal pain and distention in 78% of patients and acute lower gastrointestinal bleeding in 35%. Abdominal radiographs varied in spectrum from a nonspecific bowel gas pattern to frank right colonic pneumatosis intestinalis. Thirty-three patients received chemotherapy within 30 days before onset of abdominal symptoms. All patients were febrile (greater than 38.5 degrees C), and 33 received broad-spectrum antibiotics. Three patients received amphotericin B. Premortem, 84% of organisms cultured from blood were bacterial whereas 16% were fungal. Fungal pathogens accounted for 53% of new microorganisms seen at autopsy. Postmortem examination showed typhlitis in the following anatomic distributions: (1) confined to the cecum; (2) involving the cecum and ileum; (3) involving the cecum, ileum, and ascending colon; or (4) involving the cecum, with sporadic ulcers throughout the intestine. This review includes clinical and postmortem features of typhlitis and current strategies for diagnosis and management.

Adolescent↗
PubMed1990

Value of the postmortem examination in a pediatric population with leukemia.

Because of concerns about the declining autopsy rate, an attempt was made to evaluate the contributions from the postmortem examination in a pediatric population with leukemia. Accordingly, 161 autopsies performed between 1970 and 1985 were reviewed and the diagnoses compared with those listed in the clinical records of the same patients. New diagnoses were grouped into diagnoses of diseases thought to have contributed to the death of the patient, those of diseases thought to have contributed to the morbidity of the patient, those of presumed toxic reactions to drug therapy, and diagnoses of academic interest. The newly diagnosed diseases thought to have contributed to the patient's death either had been totally unsuspected by the clinician or had been suspected but incorrectly diagnosed. The most common revelation was the identification of mycotic infections that were thought by the clinician to have been bacterial in origin. The clinical diagnosis of these kinds of infections progressively improved during the study period. Because the changes in diagnoses and therapy, particularly the increasing use of antimycotic therapy, could be directly attributed to autopsy findings and, more recently, because of the expanding use of more toxic multiagent chemotherapy, we believe that the postmortem examination remains an important procedure, even in clinical situations where much is known about the patient.

Acute Disease↗
PubMed1988

Expanding the spectrum of the Perlman syndrome.

We report on an infant with manifestations of Perlman syndrome including polyhydramnios, macrosomia, bilateral nephromegaly with nephroblastomatosis, visceromegaly and cryptorchidism. Other findings in this infant not seen in previous patients were diaphragmatic hernia, interrupted aortic arch, hypospadias and polysplenia. This infant meets the diagnostic criteria for Perlman syndrome, suggesting that diaphragmatic hernia and cardiac defects may be additional findings in this disorder.

Abnormalities, Multiple↗