Activity-based costing methodology as tool for costing in hematopathology laboratory.

BACKGROUND: Cost analysis in laboratories represents a necessary phase in their scientific progression. AIM: To calculate indirect cost and thus total cost per sample of various tests at Hematopathology laboratory (HPL). SETTINGS AND DESIGN: Activity-based costing (ABC) method is used to calculate per cost test of the hematopathology laboratory. MATERIAL AND METHODS: Information is collected from registers, purchase orders, annual maintenance contracts (AMCs), payrolls, account books, hospital bills and registers along with informal interviews with hospital staff. RESULTS: Cost per test decreases as total number of samples increases. Maximum annual expense at the HPL is on reagents and consumables followed by manpower. Cost per test is higher for specialized tests which interpret morphological or flow data and are done by a pathologist. CONCLUSIONS: Despite several limitations and assumptions, this was an attempt to understand how the resources are consumed in a large size government-run laboratory. The rate structure needs to be revised for most of the tests, mainly for complete blood counts (CBC), bone marrow examination, coagulation tests and Immunophenotyping. This costing exercise is laboratory specific and each laboratory needs to do its own costing. Such an exercise may help a laboratory redesign its costing structure or at least understand the economics involved in the laboratory management.

Clinico-hematological profile in biphenotypic acute leukemia.

BACKGROUND: We present a clinico-hematological profile and treatment outcome of Biphenotypic Acute Leukemia (BAL). AIM: Study incidence and subtypes of BAL, correlate with age, morphology, and cytogenetic findings and correlate the clinico-hematological data with the treatment response. St Jude's and the EGIL's criteria have been compared for their diagnostic and clinical relevance. MATERIAL AND METHODS: Diagnosis was based on WHO classification, including clinical details, morphology, cytochemistry, immunophenotyping, and molecular genetics. We included those cases, which fulfilled the European Group for the Immunological Characterization of Acute Leukemia's (EGIL's) scoring system criteria for the diagnosis of BAL, as per recommendation of the WHO classification. RESULTS: There were 32 patients diagnosed with BAL, based on EGIL's criteria. Incidence of BAL was 1.2%. B-Myeloid (14 cases) followed by T-Myeloid BAL (13 cases) were the commonest subtypes. Polymorphous population of blasts (16 cases) was commonly associated with T-Myeloid BAL (10 cases). BCR ABL fusion positivity was a common cytogenetic abnormality (seven cases). Fifteen patients received chemotherapy; eight achieved complete remission (CR) at the end of the induction period. CONCLUSIONS: Pediatric BAL and T-B lymphoid BAL have a better prognosis. A comprehensive panel of reagents is required, including cytoplasmic markers; to diagnose BAL. St Jude's criteria is a simple, easy, and cost-effective method to diagnose BAL. The outcome-related prognostic factors include age, HLA-DR, CD34 negativity, and subtype of BAL. BCR-ABL expression is an important prognostic factor, as these cases will be labeled as Chronic myeloid leukemia (CML) in blast crisis with biphenotypic expression and treated accordingly.

Immunophenotypic profile of acute leukemia: critical analysis and insights gained at a tertiary care center in India.

BACKGROUND: To analyze the spectrum of various types and subtypes of acute leukemia. METHODS: Two thousand five hundred and eleven consecutive new referral cases of acute leukemia (AL) were evaluated based on WHO classification. RESULTS: It included 1,471 cases (58%) of acute lymphoblastic leukemia (ALL), 964 cases (38%) of acute myeloid leukemia (AML), 45 cases (1.8%) of chronic myelogenous leukemia in blast crisis (CMLBC), 37 cases (1.5%) of biphenotypic acute leukemia (BAL), 1 case of Triphenotypic AL, and 2 cases of acute undifferentiated leukemia (AUL). Common subtypes of ALL were B-cell ALL (76%), which comprised of intermediate stage/CALLA positive (73%), early precursor/proBALL (3%). T-cell ALL constituted 24% (351 cases) of ALL. Common subtypes of AML included AMLM2 (27%), AMLM5 (15%), AMLM0 (12%), AMLM1 (12%), APML (11%), and AML t(8;21) (9%). CMLBC was commonly of myeloid blast crisis subtype (40 cases). CONCLUSION: B-cell ALL was the commonest subtype in children and AML in adults. Overall incidence of AML in adults was low (53% only). CD13 was most sensitive and CD117 most specific for determining myeloid lineage. A minimal primary panel of nine antibodies consisting of three myeloid markers (CD13, CD33, and CD117), B-cell lymphoid marker (CD19), T-cell marker (CD7), with CD45, CD10, CD34, and HLADR could assign lineage to 92% of AL. Cytogenetics findings lead to a change in the diagnostic subtype of myeloid malignancy in 38 (1.5%) cases.

Bone marrow morphological changes in patients of chronic myeloid leukemia treated with imatinib mesylate.

BACKGROUND: Imatinib mesylate has shown promising results in chronic myeloid leukemia (CML) in all phases. This drug is an effective treatment for patients with CML in chronic phase as it induces hematological remission in nearly all patients and cytogenetic responses in many. The bone marrow changes produced by this drug are different from the treatment modalities used earlier in CML. MATERIALS & METHODS: We studied 80 patients of CML on treatment with Imatinib at doses of 400-800 mg per day. Morphological and cytogenetic evaluation (Ph analysis) of bone marrow aspirates was done at six months of treatment. RESULT: In our study, 95% (76 out of 80) patients showed complete hematological response and 63.3% showed major cytogenetic response at the end of six months of treatment. The most commonly observed changes in the bone marrow aspirates at the end of six months of therapy were in the form of reduction in the cellularity, reduction in the M: E ratio to a mean of 2:1, presence of relative erythroid hyperplasia, normalization of megakaryocytic morphology and variable increase in the bone marrow lymphocytes. None of these changes had significant correlation with the patient's Ph status. CONCLUSION: We advise study of trephine biopsies to overcome the often-faced problem of hemodiluted aspirates in these cases and evaluation of sequential bone marrows to check the durability of these morphological changes and their correlation with the cytogenetic response with emphasis on cytogenetic changes other than Ph positivity.

Activity and toxicity of 2-CDA in Langerhans cell histiocytosis: a single institutional experience.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disorder characterized by clonal proliferation of immature and abnormal bone marrow derived langerhans cells. Treatment is usually multimodal. Potent anti-monocyte as well as immunomodulatory activity of 2-CDA and its proven efficacy in many lymphoproliferative disorders has made 2-CDA a rational choice in treatment of LCH. AIM: To evaluate the efficacy and toxicity profile of 2-CDA in children with relapsed or refractory LCH. SETTING AND DESIGN: This is a pilot study and we present the initial data of the first seven patients treated at our institution. MATERIALS AND METHODS: Seven patients of relapsed and refractory LCH were enrolled from July 2000 to June 2004. The cohort of seven patients included six males and one female with a median age at initiation of cladribine was 2.25 years (range, 1.67 to 7.0 years). Three patients had received one prior chemotherapy regimen while the rest were heavily pretreated. Cladribine was administered over two hours IV daily for five days and repeated every four weeks. RESULTS: After a median of six courses of cladribine (range, 2 to 9), two (33%) patients achieved PR and two (33%) patients have SD on imaging but are clinically better. None experienced grade 3 or 4 hematologic toxicity. At a median follow-up of 19 months (range, 8 to 52 months), five patients remain alive and one patient has died. CONCLUSION: Our study shows that single agent 2-CDA is active and well-tolerated in children with relapsed or refractory LCH.

[Binge eating disorder in a population of morbid obese candidates to bariatric surgery at the Oswaldo Cruz University Hospital in Recife, PE]. / Transtorno da compulsão alimentar periódica em uma população de obesos mórbidos candidatos a cirurgia bariátrica do Hospital Universitário Oswaldo Cruz, em Recife--PE.

OBJECTIVES: To analyze the frequency of binge eating disorder (BED) and of the main psychiatric disorders associated with morbid obesity in individuals on the waiting list for bariatric surgery. METHOD: Cross sectional study. Interviews with patients from the Surgery for Obesity Program of Oswaldo Cruz University Hospital were conducted evaluating socio-demographic profile, quality of life (SF-36 scale), BED (Binge Eating Scale BES) and psychiatric disorders (M.I.N.I./DSM-IV). RESULTS: 67 out of 400 patients enrolled in the program were interviewed (16.8%). The BMI varied from 36.1 to 81.8 kg/m(2) (average 48.5 +/- 8.8). All have associated diseases, the most frequent being systemic arterial hypertension, sleeping disorders and osteopathies. The most frequent psychiatric disorders were: 47.8% generalized anxiety disorder, 29.9% major depressive disorder, single episode, 34.3% recurrent major depressive disorder. In this group 56.7% showed BED (25.4% moderate and 31.3% severe) and the worse scores in all the domains of quality of life (SF-36 scale). CONCLUSIONS: High prevalence of BED. The compulsive eaters showed a higher number of obesity treatments, higher prevalence of actual major depression, and the worse scores in all the domains of the SF-36 scale. Considering the ample range of psychopathology associated with BED and the greater probability of jeopardizing the surgery results it is very important to improve the detection of these disorders in order to provide adequate treatment.

Transtorno da compulsão alimentar periódica em uma população de obesos mórbidos candidatos a cirurgia bariátrica do Hospital Universitário Oswaldo Cruz, em Recife - PE / Binge eating disorder in a population of morbid obese candidates to bariatric surgery at the Oswaldo Cruz University Hospital in Recife, PE / Binge eating disorder in a population of morbid obese candidates to bariatric surgery at the Oswaldo Cruz University Hospital in Recife, PE

OBJETIVOS: Avaliar a freqüência de transtorno da compulsão alimentar periódica (TCAP) e dos principais transtornos psiquiátricos associados à obesidade mórbida em indivíduos à espera de cirurgia bariátrica. MÉTODOS: Estudo de corte transversal. Foram entrevistados pacientes do programa de cirurgia da obesidade do Hospital Universitário Oswaldo Cruz e avaliados o perfil sócio-demográfico, a qualidade de vida (escala SF-36), o TCAP (Binge Eating Scale ­ BES) e os transtornos psiquiátricos (Mini International Neuropsychiatry Interview ­ M.I.N.I./DSM-IV). RESULTADOS: Dos 400 pacientes inscritos no programa, 67 (16,8 por cento) foram entrevistados. O IMC variou de 36,1 a 81,8 kg/m² (média 48,5 ± 8,8). Todos os entrevistados apresentavam doenças associadas, sendo a HAS, os distúrbios do sono e as osteopatias as mais freqüentes. Os transtornos psiquiátricos mais freqüentes foram: 47,8 por cento transtorno de ansiedade generalizada; 29,9 por cento depressão atual e 34,3 por cento depressão no passado. Verificou-se TCAP em 56,7 por cento dos pacientes (25,4 por cento TCAP moderado e 31,3 por cento, grave) e esses apresentaram os piores escores em todos os domínios de qualidade de vida da escala SF-36. CONCLUSÕES: Constatou-se elevada prevalência de TCAP. Grupo com TCAP apresentou maior número de tratamentos realizados com objetivo de perder peso, elevada prevalência de depressão maior no momento da avaliação, piores escores em todos os domínios da escala de qualidade de vida SF-36. Visto que os portadores de TCAP apresentam vasta psicopatologia e maior probabilidade de comprometimento nos resultados da cirurgia, deve-se aprimorar a detecção desses distúrbios a fim de proporcionar-lhes o tratamento adequado.

OBJECTIVES: To analyze the frequency of binge eating disorder (BED) and of the main psychiatric disorders associated with morbid obesity in individuals on the waiting list for bariatric surgery. METHOD: Cross sectional study. Interviews with patients from the Surgery for Obesity Program of Oswaldo Cruz University Hospital were conducted evaluating socio-demographic profile, quality of life (SF-36 scale), BED (Binge Eating Scale ­ BES) and psychiatric disorders (M.I.N.I./DSM-IV). RESULTS: 67 out of 400 patients enrolled in the program were interviewed (16.8 percent). The BMI varied from 36.1 to 81.8 kg/m² (average 48.5 ± 8.8). All have associated diseases, the most frequent being systemic arterial hypertension, sleeping disorders and osteopathies. The most frequent psychiatric disorders were: 47.8 percent generalized anxiety disorder, 29.9 percent major depressive disorder, single episode, 34.3 percent recurrent major depressive disorder. In this group 56.7 percent showed BED (25.4 percent moderate and 31.3 percent severe) and the worse scores in all the domains of quality of life (SF-36 scale). CONCLUSIONS: High prevalence of BED. The compulsive eaters showed a higher number of obesity treatments, higher prevalence of actual major depression, and the worse scores in all the domains of the SF-36 scale. Considering the ample range of psychopathology associated with BED and the greater probability of jeopardizing the surgery results it is very important to improve the detection of these disorders in order to provide adequate treatment.

Outcome of acute myeloid leukaemia in adults: a retrospective analysis.

BACKGROUND: There are little data from India on the management of acute myeloid leukaemia. With better understanding of the biology of the disease, and routine use of high-dose cytarabine as post-remission therapy with or without haematopoietic blood stem cell transplantation (HSCT), the results have improved in the past two decades. We analysed our results in a cohort of recently treated patients. METHODS: A total of 166 newly diagnosed patients with AML (excluding acute promyelocytic leukaemia), 15-60 years of age were treated with daunorubicin (60 mg/m2/day x3 days) or idarubicin (12 mg/m2/day x3 days) with cytarabine (100 mg/m2/day continuous i.v. infusion x7 days) induction chemotherapy. Post-remission therapy included 2 cycles of high-dose cytarabine (15-18 g/m2) followed by monthly cycles of outpatient maintenance chemotherapy x4 cycles, consisting of daunorubicin (45 mg/m2 i.v. x1 day and cytarabine 100 mg/ m2 s.c. twice daily x5 days). Six patients in remission received sibling donor allogeneic HSCT. RESULTS: Morphological complete remission was achieved in 69.9% of the patients. Resistant disease after induction chemotherapy was seen in 14.6% and early mortality occurred in 16%. Relapse-free survival and event-free survival at a median of 36 months was 34% and 22%, respectively. Relapse occurred in 43.9%. The median duration of remission was 12 months. CONCLUSIONS: Our results conform to the published literature from larger cooperative studies from the West. Currently available cytotoxic drugs are unlikely to improve the results any further.

Haematologic and immunophenotypic profile of acute myeloid leukemia: an experience of Tata Memorial Hospital.

OBJECTIVES: To study the hematologic and immunophenotypic profile of 260 cases of acute myeloid leukemia at diagnosis. MATERIAL AND METHODS: This is a retrospective analysis of 260 cases of AML diagnosed at our institution between 1998 and 2000. Diagnosis was based on peripheral blood and bone marrow examination for morphology cytochemistry and immunophenotypic studies. SPSS software package, version 10, was used for statistical analysis. RESULTS: Seventy-six percent of our cases were adults. The age of the patients ranged from one year to 78 years with a median age of 27.2 years. There were 187 males and 73 females. The commonest FAB subtype, in both children and adults, was AML-M2. The highest WBC counts were seen in AML-M1 and the lowest in AML-M3 (10-97 x 10(9)/L, mean 53.8 x 10(9)/L). The mean values and range for hemoglobin was 6.8 gm/l (1.8 gm/l to 9.2 gm/l), platelet count 63.3 x 10(9)/L (32-83 x 10(9)/L), peripheral blood blasts 41.4% (5 to 77%) and bone marrow blasts 57.6% (34-96%). Myeloperoxidase positivity was highest in the M1, M2 and M3 subtypes. CD13 and CD33 were the most useful markers in the diagnosis of AML. CD14 and CD36 were most often seen in monocytic (38%) and myelomonocytic (44%) leukemias. Lymphoid antigen expression was seen in 15% of cases. CD7 expression was the commonest (11%). CONCLUSION: AML accounted for 39.8% of all acute leukemias at this institution. The most common subtype was AML-M2. Myeloperoxidase stain was a useful tool in the diagnosis of myeloid leukemias. CD13 and CD33 were the most diagnostic myeloid markers.

Leukemia in infants.

OBJECTIVE: Acute Leukemia is rare in infants. It is characterized by non-specific symptomatology requiring a high index of suspicion on the part of a pediatrician for referral and diagnosis. It has peculiar biological features, unresponsiveness to treatment and poor prognosis. METHODS: Eighteen infants with acute leukemia were seen during 1994 to 2001 and were analyzed on the basis of clinical and laboratory data. There were 13 cases of Acute Lymphoblastic Leukemia (ALL), 4 cases of Acute Myeloid Leukemia (AML) and one case remained unclassifiable, as the surface markers could not be done. Morphologically 9/13 cases of ALL were of FAB L1 type and remaining of L2 subtype, and 2/4 cases of AML were of FAB M1 type and remaining of M2 subtype. RESULT: Clinical data was available completely only in 11 cases. Hyperleucocytosis was present in 4 cases, organomegaly in 8 cases and lympadenopathy in 5 cases. One patient presented with a chloroma in the retrorbital region although there was no parenchymal involvement of the brain. Immunophenotyping could be done in 13 cases, where 7 cases were diagnosed as CALLA positive-ALL (HLADR+, CD19+, CD10+), 2 cases as Early Pre-B ALL (HLADR+, CD19+, CD10 negative), one as T ALL (cCD3+, CD2+, CD7+) and 3 cases as AML (CD13+, CD33+, HLADR+). None of our patients received treatment.

2-CdA in the treatment of hairy cell leukaemia.

INTRODUCTION: Hairy cell leukaemia (HCL) is a rare lymphoproliferative disorder. Treatment options available are splenectomy, interferon, DCF and 2-CdA. 2-CdA is considered to have curative potential as proved by the other studies. METHODS: We gave 2-CdA in a dose of 0.09/kg/day as a continuous infusion in sixteen patients of hairy cell leukaemia. RESULTS: Three patients developed neutropenia post transfusion. At the end of three months all patients were in remission. Two patients relapsed at the median follow-up of 15 months. CONCLUSION: 2-CdA in HCL can achieve complete remission, prolonged survival and care as well.

Primary Ewing's sarcoma of the cranium.

OBJECTIVE: We analyzed the data for a series of 14 patients with primary Ewing's sarcomas of the cranium who were treated since 1985. Our aim was to assess the long-term outcomes and the selection of appropriate treatment methods. METHODS: The patients were reviewed with respect to their clinical presentations, treatment, and outcomes. Computed tomographic scanning of the brain was performed for all patients. Skeletal surveys with routine radiographs and technetium-99 bone scans to detect extracranial Ewing's sarcomas were performed for all patients. For all 14 patients, radical tumor excision was achieved surgically. All patients were then subjected to adjuvant multidrug chemotherapy and radiotherapy. The follow-up periods ranged from 8 months to 8 years (mean, 4.25 yr). RESULTS: The predominant presenting features were headaches, increased intracranial pressure, and scalp swelling. Excision was nearly total for nine patients and total for five patients. All patients experienced uneventful postoperative courses. One patient experienced a local recurrence, which was detected 2 years after surgery. This recurrent tumor was completely excised, and additional chemotherapy was administered. Eight of the 14 patients (57.1%) studied have survived 5 years or longer. CONCLUSION: Although primary Ewing's sarcoma of the cranium is a malignant bone tumor, it is associated with a good prognosis when treated with radical surgery, aggressive multidrug chemotherapy, and radiotherapy.

Acute lymphoblastic leukemia in India: an analysis of prognostic factors using a single treatment regimen.

BACKGROUND: In the past, treatment results in Indian children with ALL have been poor, primarily due to inadequate chemotherapy and supportive care, but perhaps reflecting differences from Western countries in the pattern of subtypes. In an attempt to improve survival, we have used a more intensive treatment protocol, MCP841, and examined prognostic factors. PATIENTS AND METHODS: Five hundred thirty previously untreated patients < 25 years of age with ALL were entered on study at the Tata Memorial Hospital, Mumbai. Treatment consisted of three successive induction cycles, consolidation and six maintenance cycles. CNS prophylactic therapy consisted of cranial irradiation (2000 cGy) for patients above two years and high-dose cytarabine for patients less than two years. The total treatment duration was two years. RESULTS: Most patients had hepatosplenomegaly (80%) and or lymphadenopathy (79%) and 21% were of T-cell immunophenotype, but very few (1.3%) had CNS disease. CR was achieved in 484 (91.3%) patients and 145 (29.9%) patients relapsed. There were 36 induction deaths and 49 remission deaths, but the toxic death rate was significantly lower after 1990. In patients treated since 1990, three risk groups could be discerned: 1) WBC < 60,000 per mm3 and no lymphadenopathy (77% event-free survival (EFS) at five years): 2) WBC < 60,000 per mm3 with lymphadenopathy (53% EFS) or, WBC > 60,000 per mm3 and Hb 6 gm/dl or above (48% EFS): and 3) WBC > 60,000 per mm3 and Hb below 6 gm dl (16% EFS). In a multivariate model, only WBC, Hb and lymphadenopathy were significantly associated with EFS (P < 0.01). CONCLUSIONS: The CR and EFS rates achieved represent a significant improvement over previous results at this institution. Bulky extramedullary disease was an important risk factor in this series, but age and WBC alone inadequately defined risk groups, suggesting that prognostic factors may vary in different world regions.

Sezary cells with hairy projections.

A case of sezary syndrome where the sezary cells showed cytoplasmic hairy projections is reported. The patient had typical exfoliative erythematous dermatitis, high white cell count, atypical lymphocytes of T-phenotype with folded nuclei and bone marrow involvement. The ultra structure study showed cerebriform nucleus and cytoplasmic projections.

Acute promyelocytic leukemia: all-trans retinoic acid (ATRA) along with chemotherapy is superior to ATRA alone.

This study was conducted to compare the results of treatment of acute promyelocytic leukemia (APL) with all-trans retinoic acid alone (ATRA) or a combination therapy of ATRA followed by chemotherapy. Forty-three patients treated between February 1992 and February 1996 were included in this study. Eighteen patients were treated with ATRA alone and 25 patients were treated with ATRA followed by chemotherapy. The cytogenetic analysis was done in 41 patients at presentation, following treatment, and at follow-up. A complete response (CR) was achieved in 13 (72%) patients on ATRA and 19 (76%) on ATRA followed by chemotherapy. Eleven of 13 patients with response to ATRA alone relapsed with median survival of eight months (range, 1 to 28). One patient died of hepatitis in CR and one patient is alive 2 years after diagnosis. In the combination therapy arm, 10 patients are in CR with a median follow-up of 22 months (range, 6 to 56 months). After achieving a CR, four patients died due to infections during chemotherapy therapy, and only 5 of 19 patients have relapsed. Major cytogenetic response was seen in 8 of the 10 patients in whom cytogenetic data was available after treatment with ATRA at the time of remission. Similarly, 13 of 15 for whom data was available showed a major cytogenetic response after treatment with ATRA plus chemotherapy. Prior to relapse, 80% of the patients had an increase in the percentage of t(15;17) cells in the marrow. Patients with a complete hematological response but no cytogenetic response relapsed within six months. Ten patients died prior to response evaluation. Two patients who received ATRA died of retinoic acid syndrome, one of pneumonia, and one of intracranial hemorrhage. Of the six patients on ATRA and chemotherapy, four died of retinoic acid syndrome (RAS), one of intracranial hemorrhage, and one of left ventricular failure. Only one patient is alive at 24 months following treatment with ATRA alone. The relapse-free survival is 42% at four years for patients treated with ATRA followed by chemotherapy. This trial is a historical comparison of ATRA alone and ATRA with subsequent combination chemotherapy. Nonetheless, the trial shows a significant improvement in the event free survival of patients receiving chemotherapy as consolidation following ATRA.

Results of allogeneic bone marrow transplant in chronic myeloid leukaemia following conditioning with busulfan and cyclophosphamide.

OBJECTIVE: To study the outcome of oral busulfan and intravenous cyclophosphamide (BuCY 2 regimen) followed by allogeneic bone marrow transplant (BMT) in a cohort of patients with Philadelphia chromosome (Ph+) chronic myeloid leukaemia (CML) in a single centre. METHODS: From 1991 to March 1998, a total of 27 consecutive Ph+ CML patients received busulfan 4 mg/kg/day over 4 days and cyclophosphamide 60 mg/kg/day over 2 days followed by infusion of HLA-identical sibling haematopoietic stem cells. All except one (who received peripheral blood stem cells) were given donor bone marrow cells. Post-transplant graft versus host disease (GVHD) prophylaxis included a short course of methotrexate (on days +1, +3, +6 and +11) and cyclosporine till day +180. RESULTS: With a median follow-up of 30.5 months (1-55+ months), 14 patients (52%) are alive free from relapse. Early mortality was relatively high with 10 patients (37%) dying within first 100 days post-transplant. Acute GVHD developed in 14 patients (52%) inspite of GVHD prophylaxis with methotrexate and cyclosporine; six had grade I/II and eight grade III/IV. Chronic GVHD developed in five of 15 patients who lived beyond 70 days. CONCLUSION: Allogeneic BMT appears to result in eradication of CML and ensure disease free survival in about half of the young patients. However, efforts should be on to minimise early mortality.

Hairy cell leukemia--results with alpha interferon therapy.

Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder. Although now multiple treatment options are being available, the optimal treatment of this disease still remains debatable. Inspite of the advent of newer purine analogues, in India recombinant interferon is the only freely available first line treatment. We report our experience of long term remissions in HCL with interferon alpha 2a. Of a total of 35 cases of HCL we were able to treat 19 cases with interferon. Of 18 evaluable cases an overall response of 88.9% was achieved. With a median follow up of 31 months a disease free survival was 83%. Thus with a dose of 3 million units s.c. daily for 6 months at least, we feel that a reasonably good long term remission can be obtained. The cost of the treatment however, is still a deterrent.

Retinoblastoma: problems and perspectives from India.

This study examined the salient clinical and epidemiological characteristics of retinoblastoma (RB) in India, thereby highlighting the problems encountered there. The epidemiological characteristics of 296 patients with RB over 8 years were evaluated using hospital records and postal follow-ups. Unilateral disease was seen in 61.8% of patients. The overall median age at presentation was 3.5 years (3.5 years for unilateral RB and 1.0 years for bilateral RB). The male/female ratio was 1.4:1. The median duration of symptomatic disease was 8 months. Consanguineous marriage was seen in 17% and family history of RB was noted in 1.7% cases. Also, 2% had a history of other malignancy in the family. Associated congenital malformation was seen in 10.5% of cases. A second malignancy was seen in 0.67% of cases at a mean duration of 4.5 years after completion of therapy. A predominance of advanced-stage disease (74.5% had Reese-Ellsworth group IV and V disease) was seen in our series. Only 43.6% of patients had disease localized to the globe without any infiltration/invasion. The majority of cases had advanced-stage disease at presentation and came from the underprivileged class of society. Patients with bilateral RB presented much earlier than those with unilateral disease. In patients with unilateral RB, higher age at presentation as well as advanced disease may be related to much delay in seeking medical attention. In view of the advanced stage at presentation, there also exist a possibility of difference in the biology of the tumor seen in these patients.

Translocation (11;14)(q13;q32) and overexpression of cyclin D1 protein in a CD23-positive low-grade B-cell neoplasm.

We describe a case of low-grade B-cell neoplasm with features overlapping between B-chronic lymphocytic leukemia (CLL) and mantle cell lymphoma (MCL). The patient presented with a 10-year history of stable CLL without any treatment. The peripheral-blood picture was consistent with atypical mixed CLL (French-American-British criteria), whereas the lymph-node histology was more consistent with MCL. Neoplastic cells were strongly positive for surface immunoglobulin M, kappa, CD5, CD20, CD23, and cyclin D1. Expression of CD11c was weak. Translocation (11;14) and der(10)t(10;?)(p11;?) were the primary cytogenetic changes observed in both peripheral blood (47%) and lymph node (7%). Trisomy 12 was absent. Deletion 6q21 and rearrangements involving 1p/q, consistently associated with progression in lymphomas, also were noted in the peripheral blood but were nonclonal. The present case and similar cases with features overlapping between CLL and MCL most likely represent hybrids. In cases with features of typical CLL, t(11;14) is probably associated with gradual progression and may precede clinical and histologic transformation.