Background: Acute promyelocytic leukaemia (APL) usually presents with disseminated intravascular ... more Background: Acute promyelocytic leukaemia (APL) usually presents with disseminated intravascular coagulation or hyper-fibrinogenolysis followed by bleeding manifestation. But thrombosis as a presenting manifestation is less often reported in APL. Objectives: We describe an uncommon case of chronic deep venous thrombosis (DVT) of lower limb with unsuspected APL. Results: A 27 year old male presented with DVT and was treated with enoxaparin and later Dabigatran for 2 months without any improvement. A routine haematological assessment showed Leucopenia with few circulating abnormal promyelocytes. The bone marrow assessment with flow cytometry and molecular studies established the diagnosis of APL. Patient was never having any bleeding manifestation despite on anticoagulation too. A thrombophilia work up revealed presence of hyper-homocysteinemia and mild lupus anticoagulant in addition to APL as a prothrombotic event. Conclusion: DVT with leukopenia warrants further investigation to ru...
Children with underlying cancer are often immunocompromised. Data on severity of coronavirus dise... more Children with underlying cancer are often immunocompromised. Data on severity of coronavirus disease 2019 (COVID-19) in children with cancer and its outcomes is emerging. Treatment protocols of specific cancers are decided based on the infrastructure, availability of supportive-care, and logistic issues of the locality. The purpose of the study was clinical analysis of COVID-19 in children and adolescents with cancer. The retrospective observational study was conducted at a tertiary healthcare-center in East India. Children and adolescents (aged 0-19 years) with cancer and under treatment with reverse-transcriptase-polymerase-chain-reaction (RT-PCR) confirmed COVID-19 between 5-July-2020 and 5-December-2020 were studied. Median age of the 68 identified patients was six years. Acute leukemia was the most common (66%) diagnosis. COVID-19 was asymptomatic/mildly symptomatic in 91% and moderate to severe in only 9% of patients. Fever (87%) was the commonest symptom, followed-by cough/coryza (75%). Three patients with acute myeloid leukemia (AML) and severe/critical COVID-19 and associated neutropenic sepsis were required transfer to the intensive-care-unit (ICU) for management. Three (4.4%) patients succumbed with COVID-19. Delay in treatment was observed in 63.2% of patients, and the median duration of delay was 28 days after acquiring COVID-19. Median time to attain negative COVID-19 RT-PCR was 16 days, and eight patients were repeat positives. While pediatric and adolescent cancer patients on active treatment may have a higher risk of mortality from severe COVID-19 than their healthy counterparts, the risk may be much lower than deemed. It is essential to continue cancer therapy in these children. Delay in treatment remains a concern.
Acquired pure red cell aplasia (PRCA) following use of recombinant erythropoietin (rEPO) is disti... more Acquired pure red cell aplasia (PRCA) following use of recombinant erythropoietin (rEPO) is distinctly rare and sporadically reported in the literature. We discuss a case of PRCA following the usage of rEPO (darbepoetin-α) during the management of anemia of chronic kidney disease in an elderly male subject with review of literature and a brief insight into proposed pathophysiologic mechanism, diagnosis, and management.
Introduction: Acute promyelocytic leukemia (APL) is one of the hematological emergencies requirin... more Introduction: Acute promyelocytic leukemia (APL) is one of the hematological emergencies requiring early detection and treatment. The hematology laboratory plays a pivotal role in management. The correct morphological identification of APL is made even before the confirmation by the cytogenetic or molecular analysis. Automated hematology analyzers are the backbone of the hematology laboratories. Other than providing routine counts and differentials, they are also capable of producing sufficient information in the form of leucocyte cell population data (CPD) scatter plots depicting the leucocyte subpopulation. These data are primarily underutilized, particularly in assessing the lineage of various hematopoietic neoplasm. Here we analyzed the utility of leucocyte CPD and the pattern of scatter plot in Sysmex XN 1000 analyzer for the detection of acute promyelocytic leukemia. Materials and Results: We included 100 controls and 100 AML cases. Leucocyte CPD parameters were compared along...
Aim: This study aims to review the current literature and to focus on etiopathogenesis, clinical ... more Aim: This study aims to review the current literature and to focus on etiopathogenesis, clinical profile, diagnosis, and treatment of extranodal nasofacial natural killer (NK)/T-cell lymphoma. Materials and Methods: It is based upon the available literatures from PubMed, Scopus, and Google scholar with the keywords: etiopathogenesis, clinical pictures, diagnostic methods, and current treatment of extranodal nasofacial NK/T-cell lymphoma from 2002 to 2017. Results: Primary nasofacial lymphoma is a rare form of malignancy in head and neck area. Extranodal nasofacial NK/T-cell lymphoma is an unusual clinical entity, which is an aggressive entity of non-Hodgkin's lymphoma with distinct clinicopathological pictures. It is possibly associated with Epstein–Barr virus infection. It is highly aggressive disease with poor prognosis. Nasofacial NK/T-cell lymphoma or lethal midline granuloma is often associated with destruction of midface and surrounding areas such as orbit, paranasal sinuses, and palate. The clinical picture is highly variable, often missed by clinician and depends on location and histopathological type of the lesion. Histopathological and immunohistochemistry are important tools for diagnosis of nasofacial NK/T-cell lymphoma. Histopathological picture shows angiocentric and angiodestructive pattern of tumor cells which often mimic vasculitis. Radiotherapy is the treatment of choice which improves quality and longevity of life whereas addition of chemotherapy gives additional benefit to the patients. Conclusion: Practicing physicians and otorhinolaryngologist need to be aware of this nonspecific presentation of lesion to prevent delay in diagnosis. Early diagnosis and intervention prolongs the survival of the patients.
This study evaluates our institution's target trough cyclosporine (CSA) concentrations as pre... more This study evaluates our institution's target trough cyclosporine (CSA) concentrations as predictors of severe acute graft versus host disease (aGvHD) in children receiving either matched related or unrelated hematopoietic stem cell transplantation (HSCT). The outcomes of 87 consecutive children who underwent allogeneic HSCT and received CSA and methotrexate as prophylaxis against aGvHD between October 1, 1999 and September 30, 2002 were retrospectively evaluated. The proportion of time that each patient maintained a whole blood CSA concentration within or above the initial target range (105-155 ng/mL or 155-210 ng/mL) was calculated for each of the following time periods: in each week after HSCT from day 0 to +28; in the week preceding engraftment; and in the week preceding the onset of aGvHD. Patients were prospectively evaluated twice weekly for the presence and severity of aGvHD by senior attending physicians. The relationship between potential predictors and the development...
Background: Immunosuppressive therapy (IST) is the alternative first line treatment in children w... more Background: Immunosuppressive therapy (IST) is the alternative first line treatment in children with aplastic anemia (AA) who have no HLA match siblings available. The long-term outcome of patients with AA who survive after IST is unknown. We evaluated outcomes of children with AA treated with IST at long-term follow up. Methods: we retrospectively reviewed the hospital records of children with AA from 1984 to 2004, treated at our institution with horse-derived antithymocyte globulin (hATG) 160 mg/Kg over 4 days, short course of prednisone and cyclosporine (CS). Results: Forty two patients were treated with IST (25 boys, 18 girls). The median age at diagnosis was 8.5 years. Twenty nine (69%), eight (19%) and five (12%) patients were diagnosed with severe, very severe, and moderate AA, respectively. Nine patients (21%) had hepatitis associated AA. Twenty seven patients (64%) received one course of ATG and fifteen (36%) received 2 courses (8 received 2 courses of hATG and 7 received 1...
The diagnosis of chronic lymphoproliferative disorder (CLPD) or non-Hodgkin Lymphoma (NHL) is bas... more The diagnosis of chronic lymphoproliferative disorder (CLPD) or non-Hodgkin Lymphoma (NHL) is based on the detection of the abnormal clonal lymphoid cells. The flow cytometry (FCM) immunophenotyping not only plays an essential role in the screening of CLPD but also helps in the specific identification and characterization of the expanded aberrant lymphocytes. Over decades, it has evolved from a single parameter to multi-parameter assessment by 3-to 12-color FCM. The greatest challenge is to characterize abnormal lymphoid cells by a limited immunophenotype (IPT) panel. A study was undertaken to evaluate the diagnostic usefulness of a single lymphoid screening tube (LST) FCM assays that included a multiplex of 12 antibody cocktails consisting of CD45,
Between June 1994 and December 2003, 31 patients with haematological disorders presenting at the ... more Between June 1994 and December 2003, 31 patients with haematological disorders presenting at the Hospital for Sick Children, Toronto, Canada, received a stem cell transplant (SCT) from a mismatched related donor. Diagnoses included ALL (15), AML (10), CML (1), JMML (1), SAA (2), de novo MDS (1) and Fanconi anaemia (1). Patients were aged between 0.9 to 17.8 years (mean 8.5 years). There were 20 males and 11 females. HLA typing included A, B and DRB1 on all patients (Class 1 alleles by low resolution molecular technique and Class 2 by high resolution technique). 22 patients had 1 major mismatch and 9 had 2–3 major mismatches. The source of stem cells was bone marrow in 15 patients (mean total nucleated cell dose 4.44 x 108/kg, range 1.89–6.63), peripheral blood (PBSC) in 15 patients (mean CD34 dose 11.80 x 106/kg, range 3.19–42.18) and one patient received both BM and PBSC. Of those who received PBSC, 9 had CD34 selection, 3 had CD34 selection and T-cell depletion, and 3 received an ...
The clinical characteristics and outcome of 15 patients with acute myelogenous leukemia (AML) who... more The clinical characteristics and outcome of 15 patients with acute myelogenous leukemia (AML) who experienced relapse at least 5 years after induction of complete remission (very late-relapse AML) are described. This subgroup represented 3% of all relapsed patients seen at this institution over the same time period. There were eight males in this cohort and the median age at diagnosis was 48 years (range 13 - 77 years). Nine patients had M4/M5 French - American - British (FAB) classification subtype and most had intermediate risk cytogenetics. The median duration of first complete remission (CR-1) was 9 years (range 5.2 - 11.5 years). Thirteen patients (86%) achieved CR-2 with reinduction therapy. The 5-year relapse-free survival and overall survival rates of this cohort were 59% and 51%, respectively. We conclude that very late-relapse AML is a rare event, and that reinduction in these patients is associated with very high CR rates and a potential cure fraction.
Primary cardiac lymphoma (PCL) is a rare presentation of lymphoma in children. The emergency natu... more Primary cardiac lymphoma (PCL) is a rare presentation of lymphoma in children. The emergency nature of its presentation (heart failure and arrhythmias) makes management of this entity difficult. The authors describe two children with lymphoma primarily involving the heart; one patient was a case of PCL and the other was a case of lymphoma involving the heart along with pelvic involvement. These two patients, together with a literature review on the topic, suggest that if the diagnosis of PCL is made early and treatment is expeditiously started, patients may do well.
Recurrent respiratory papillomatosis is the most common neoplasm of the larynx in childhood. Exte... more Recurrent respiratory papillomatosis is the most common neoplasm of the larynx in childhood. Extension into lung parenchyma occurs in less than 1% of patients and has a low risk of malignant transformation. Treatment options for intrapulmonary spread have shown limited success. We describe a case of recurrent respiratory papillomatosis with extensive parenchymal involvement and adenosquamous carcinoma in a 14-year-old girl.
The persistence of abnormal coagulation test results after standard treatment with fresh frozen p... more The persistence of abnormal coagulation test results after standard treatment with fresh frozen plasma (FFP) poses significant problems in children with acute leukemia requiring a diagnostic lumbar puncture and intrathecal chemotherapy. We report the prophylactic use of a single dose of 90 microg/kg recombinant activated factor VII (rFVIIa) in three children and the rapid correction of abnormal coagulation test results previously not corrected by FFP. Administration of rFVIIa was useful in avoiding a delay of diagnostic lumbar punctures and intrathecal chemotherapy. Hemorrhagic complications and adverse effects of rFVIIa were not observed. Prospective evaluation of this indication and dose appears warranted. (c) 2005 Wiley-Liss, Inc.
Aplastic anemia (AA) is mediated by T-cell autoimmunity in the majority of cases; it is rare and ... more Aplastic anemia (AA) is mediated by T-cell autoimmunity in the majority of cases; it is rare and mostly idiopathic in children. We describe a child, who developed AA following Graves' disease which could not be attributed to antithyroid drugs. We hypothesized that both diseases were caused by similar autoimmune process. We monitored the blood counts and did not administer any conventional treatment for AA assuming that the existing anti- hematopoietic stem cell humoral and cellular immunity might subside with induction of remission of Grave's disease. The child went into complete remission with the treatment of the Graves' disease.
Immunosuppressive therapy (IST) is the alternative treatment in children with aplastic anemia (AA... more Immunosuppressive therapy (IST) is the alternative treatment in children with aplastic anemia (AA) who do not have an HLA-matched sibling. The aim of this study is to evaluate the outcome of children with AA treated with IST. We retrospectively reviewed the hospital records of children with AA from 1984 to 2004, treated at our institution with antithymocyte globulin (ATG), cyclosporine (CS), and short course of prednisone. Forty-two patients were treated with IST (24 boys, 18 girls); of whom 26% received G-CSF. The median age at diagnosis was 8.5 years. Sixty-nine, 19, and 12% were diagnosed with severe, very severe, and moderate AA, respectively. Twenty-one percent had hepatitis-associated AA. Median follow-up time was 53.3 months. Sixty-two percent had complete response; 19% had partial response. Two patients relapsed and received a second course of ATG; both had a partial response. The actuarial 5 years survival rate was 67.5%. Two patients developed myelodysplastic syndrome (MDS); both received long-term G-CSF and had partial response after two courses of IST. Fifteen percent of survivors had significant hypertension which persisted after CS was discontinued. This study shows promising response in children with AA treated with IST; however, the outcome was inferior to our institutional results with hematopoietic stem cell transplantation from a sibling donor. Hypertension and MDS are late complications. Longer follow-up, larger cohorts, and prospective studies are warranted to evaluate late complications and risk factors.
Background: Acute promyelocytic leukaemia (APL) usually presents with disseminated intravascular ... more Background: Acute promyelocytic leukaemia (APL) usually presents with disseminated intravascular coagulation or hyper-fibrinogenolysis followed by bleeding manifestation. But thrombosis as a presenting manifestation is less often reported in APL. Objectives: We describe an uncommon case of chronic deep venous thrombosis (DVT) of lower limb with unsuspected APL. Results: A 27 year old male presented with DVT and was treated with enoxaparin and later Dabigatran for 2 months without any improvement. A routine haematological assessment showed Leucopenia with few circulating abnormal promyelocytes. The bone marrow assessment with flow cytometry and molecular studies established the diagnosis of APL. Patient was never having any bleeding manifestation despite on anticoagulation too. A thrombophilia work up revealed presence of hyper-homocysteinemia and mild lupus anticoagulant in addition to APL as a prothrombotic event. Conclusion: DVT with leukopenia warrants further investigation to ru...
Children with underlying cancer are often immunocompromised. Data on severity of coronavirus dise... more Children with underlying cancer are often immunocompromised. Data on severity of coronavirus disease 2019 (COVID-19) in children with cancer and its outcomes is emerging. Treatment protocols of specific cancers are decided based on the infrastructure, availability of supportive-care, and logistic issues of the locality. The purpose of the study was clinical analysis of COVID-19 in children and adolescents with cancer. The retrospective observational study was conducted at a tertiary healthcare-center in East India. Children and adolescents (aged 0-19 years) with cancer and under treatment with reverse-transcriptase-polymerase-chain-reaction (RT-PCR) confirmed COVID-19 between 5-July-2020 and 5-December-2020 were studied. Median age of the 68 identified patients was six years. Acute leukemia was the most common (66%) diagnosis. COVID-19 was asymptomatic/mildly symptomatic in 91% and moderate to severe in only 9% of patients. Fever (87%) was the commonest symptom, followed-by cough/coryza (75%). Three patients with acute myeloid leukemia (AML) and severe/critical COVID-19 and associated neutropenic sepsis were required transfer to the intensive-care-unit (ICU) for management. Three (4.4%) patients succumbed with COVID-19. Delay in treatment was observed in 63.2% of patients, and the median duration of delay was 28 days after acquiring COVID-19. Median time to attain negative COVID-19 RT-PCR was 16 days, and eight patients were repeat positives. While pediatric and adolescent cancer patients on active treatment may have a higher risk of mortality from severe COVID-19 than their healthy counterparts, the risk may be much lower than deemed. It is essential to continue cancer therapy in these children. Delay in treatment remains a concern.
Acquired pure red cell aplasia (PRCA) following use of recombinant erythropoietin (rEPO) is disti... more Acquired pure red cell aplasia (PRCA) following use of recombinant erythropoietin (rEPO) is distinctly rare and sporadically reported in the literature. We discuss a case of PRCA following the usage of rEPO (darbepoetin-α) during the management of anemia of chronic kidney disease in an elderly male subject with review of literature and a brief insight into proposed pathophysiologic mechanism, diagnosis, and management.
Introduction: Acute promyelocytic leukemia (APL) is one of the hematological emergencies requirin... more Introduction: Acute promyelocytic leukemia (APL) is one of the hematological emergencies requiring early detection and treatment. The hematology laboratory plays a pivotal role in management. The correct morphological identification of APL is made even before the confirmation by the cytogenetic or molecular analysis. Automated hematology analyzers are the backbone of the hematology laboratories. Other than providing routine counts and differentials, they are also capable of producing sufficient information in the form of leucocyte cell population data (CPD) scatter plots depicting the leucocyte subpopulation. These data are primarily underutilized, particularly in assessing the lineage of various hematopoietic neoplasm. Here we analyzed the utility of leucocyte CPD and the pattern of scatter plot in Sysmex XN 1000 analyzer for the detection of acute promyelocytic leukemia. Materials and Results: We included 100 controls and 100 AML cases. Leucocyte CPD parameters were compared along...
Aim: This study aims to review the current literature and to focus on etiopathogenesis, clinical ... more Aim: This study aims to review the current literature and to focus on etiopathogenesis, clinical profile, diagnosis, and treatment of extranodal nasofacial natural killer (NK)/T-cell lymphoma. Materials and Methods: It is based upon the available literatures from PubMed, Scopus, and Google scholar with the keywords: etiopathogenesis, clinical pictures, diagnostic methods, and current treatment of extranodal nasofacial NK/T-cell lymphoma from 2002 to 2017. Results: Primary nasofacial lymphoma is a rare form of malignancy in head and neck area. Extranodal nasofacial NK/T-cell lymphoma is an unusual clinical entity, which is an aggressive entity of non-Hodgkin's lymphoma with distinct clinicopathological pictures. It is possibly associated with Epstein–Barr virus infection. It is highly aggressive disease with poor prognosis. Nasofacial NK/T-cell lymphoma or lethal midline granuloma is often associated with destruction of midface and surrounding areas such as orbit, paranasal sinuses, and palate. The clinical picture is highly variable, often missed by clinician and depends on location and histopathological type of the lesion. Histopathological and immunohistochemistry are important tools for diagnosis of nasofacial NK/T-cell lymphoma. Histopathological picture shows angiocentric and angiodestructive pattern of tumor cells which often mimic vasculitis. Radiotherapy is the treatment of choice which improves quality and longevity of life whereas addition of chemotherapy gives additional benefit to the patients. Conclusion: Practicing physicians and otorhinolaryngologist need to be aware of this nonspecific presentation of lesion to prevent delay in diagnosis. Early diagnosis and intervention prolongs the survival of the patients.
This study evaluates our institution's target trough cyclosporine (CSA) concentrations as pre... more This study evaluates our institution's target trough cyclosporine (CSA) concentrations as predictors of severe acute graft versus host disease (aGvHD) in children receiving either matched related or unrelated hematopoietic stem cell transplantation (HSCT). The outcomes of 87 consecutive children who underwent allogeneic HSCT and received CSA and methotrexate as prophylaxis against aGvHD between October 1, 1999 and September 30, 2002 were retrospectively evaluated. The proportion of time that each patient maintained a whole blood CSA concentration within or above the initial target range (105-155 ng/mL or 155-210 ng/mL) was calculated for each of the following time periods: in each week after HSCT from day 0 to +28; in the week preceding engraftment; and in the week preceding the onset of aGvHD. Patients were prospectively evaluated twice weekly for the presence and severity of aGvHD by senior attending physicians. The relationship between potential predictors and the development...
Background: Immunosuppressive therapy (IST) is the alternative first line treatment in children w... more Background: Immunosuppressive therapy (IST) is the alternative first line treatment in children with aplastic anemia (AA) who have no HLA match siblings available. The long-term outcome of patients with AA who survive after IST is unknown. We evaluated outcomes of children with AA treated with IST at long-term follow up. Methods: we retrospectively reviewed the hospital records of children with AA from 1984 to 2004, treated at our institution with horse-derived antithymocyte globulin (hATG) 160 mg/Kg over 4 days, short course of prednisone and cyclosporine (CS). Results: Forty two patients were treated with IST (25 boys, 18 girls). The median age at diagnosis was 8.5 years. Twenty nine (69%), eight (19%) and five (12%) patients were diagnosed with severe, very severe, and moderate AA, respectively. Nine patients (21%) had hepatitis associated AA. Twenty seven patients (64%) received one course of ATG and fifteen (36%) received 2 courses (8 received 2 courses of hATG and 7 received 1...
The diagnosis of chronic lymphoproliferative disorder (CLPD) or non-Hodgkin Lymphoma (NHL) is bas... more The diagnosis of chronic lymphoproliferative disorder (CLPD) or non-Hodgkin Lymphoma (NHL) is based on the detection of the abnormal clonal lymphoid cells. The flow cytometry (FCM) immunophenotyping not only plays an essential role in the screening of CLPD but also helps in the specific identification and characterization of the expanded aberrant lymphocytes. Over decades, it has evolved from a single parameter to multi-parameter assessment by 3-to 12-color FCM. The greatest challenge is to characterize abnormal lymphoid cells by a limited immunophenotype (IPT) panel. A study was undertaken to evaluate the diagnostic usefulness of a single lymphoid screening tube (LST) FCM assays that included a multiplex of 12 antibody cocktails consisting of CD45,
Between June 1994 and December 2003, 31 patients with haematological disorders presenting at the ... more Between June 1994 and December 2003, 31 patients with haematological disorders presenting at the Hospital for Sick Children, Toronto, Canada, received a stem cell transplant (SCT) from a mismatched related donor. Diagnoses included ALL (15), AML (10), CML (1), JMML (1), SAA (2), de novo MDS (1) and Fanconi anaemia (1). Patients were aged between 0.9 to 17.8 years (mean 8.5 years). There were 20 males and 11 females. HLA typing included A, B and DRB1 on all patients (Class 1 alleles by low resolution molecular technique and Class 2 by high resolution technique). 22 patients had 1 major mismatch and 9 had 2–3 major mismatches. The source of stem cells was bone marrow in 15 patients (mean total nucleated cell dose 4.44 x 108/kg, range 1.89–6.63), peripheral blood (PBSC) in 15 patients (mean CD34 dose 11.80 x 106/kg, range 3.19–42.18) and one patient received both BM and PBSC. Of those who received PBSC, 9 had CD34 selection, 3 had CD34 selection and T-cell depletion, and 3 received an ...
The clinical characteristics and outcome of 15 patients with acute myelogenous leukemia (AML) who... more The clinical characteristics and outcome of 15 patients with acute myelogenous leukemia (AML) who experienced relapse at least 5 years after induction of complete remission (very late-relapse AML) are described. This subgroup represented 3% of all relapsed patients seen at this institution over the same time period. There were eight males in this cohort and the median age at diagnosis was 48 years (range 13 - 77 years). Nine patients had M4/M5 French - American - British (FAB) classification subtype and most had intermediate risk cytogenetics. The median duration of first complete remission (CR-1) was 9 years (range 5.2 - 11.5 years). Thirteen patients (86%) achieved CR-2 with reinduction therapy. The 5-year relapse-free survival and overall survival rates of this cohort were 59% and 51%, respectively. We conclude that very late-relapse AML is a rare event, and that reinduction in these patients is associated with very high CR rates and a potential cure fraction.
Primary cardiac lymphoma (PCL) is a rare presentation of lymphoma in children. The emergency natu... more Primary cardiac lymphoma (PCL) is a rare presentation of lymphoma in children. The emergency nature of its presentation (heart failure and arrhythmias) makes management of this entity difficult. The authors describe two children with lymphoma primarily involving the heart; one patient was a case of PCL and the other was a case of lymphoma involving the heart along with pelvic involvement. These two patients, together with a literature review on the topic, suggest that if the diagnosis of PCL is made early and treatment is expeditiously started, patients may do well.
Recurrent respiratory papillomatosis is the most common neoplasm of the larynx in childhood. Exte... more Recurrent respiratory papillomatosis is the most common neoplasm of the larynx in childhood. Extension into lung parenchyma occurs in less than 1% of patients and has a low risk of malignant transformation. Treatment options for intrapulmonary spread have shown limited success. We describe a case of recurrent respiratory papillomatosis with extensive parenchymal involvement and adenosquamous carcinoma in a 14-year-old girl.
The persistence of abnormal coagulation test results after standard treatment with fresh frozen p... more The persistence of abnormal coagulation test results after standard treatment with fresh frozen plasma (FFP) poses significant problems in children with acute leukemia requiring a diagnostic lumbar puncture and intrathecal chemotherapy. We report the prophylactic use of a single dose of 90 microg/kg recombinant activated factor VII (rFVIIa) in three children and the rapid correction of abnormal coagulation test results previously not corrected by FFP. Administration of rFVIIa was useful in avoiding a delay of diagnostic lumbar punctures and intrathecal chemotherapy. Hemorrhagic complications and adverse effects of rFVIIa were not observed. Prospective evaluation of this indication and dose appears warranted. (c) 2005 Wiley-Liss, Inc.
Aplastic anemia (AA) is mediated by T-cell autoimmunity in the majority of cases; it is rare and ... more Aplastic anemia (AA) is mediated by T-cell autoimmunity in the majority of cases; it is rare and mostly idiopathic in children. We describe a child, who developed AA following Graves' disease which could not be attributed to antithyroid drugs. We hypothesized that both diseases were caused by similar autoimmune process. We monitored the blood counts and did not administer any conventional treatment for AA assuming that the existing anti- hematopoietic stem cell humoral and cellular immunity might subside with induction of remission of Grave's disease. The child went into complete remission with the treatment of the Graves' disease.
Immunosuppressive therapy (IST) is the alternative treatment in children with aplastic anemia (AA... more Immunosuppressive therapy (IST) is the alternative treatment in children with aplastic anemia (AA) who do not have an HLA-matched sibling. The aim of this study is to evaluate the outcome of children with AA treated with IST. We retrospectively reviewed the hospital records of children with AA from 1984 to 2004, treated at our institution with antithymocyte globulin (ATG), cyclosporine (CS), and short course of prednisone. Forty-two patients were treated with IST (24 boys, 18 girls); of whom 26% received G-CSF. The median age at diagnosis was 8.5 years. Sixty-nine, 19, and 12% were diagnosed with severe, very severe, and moderate AA, respectively. Twenty-one percent had hepatitis-associated AA. Median follow-up time was 53.3 months. Sixty-two percent had complete response; 19% had partial response. Two patients relapsed and received a second course of ATG; both had a partial response. The actuarial 5 years survival rate was 67.5%. Two patients developed myelodysplastic syndrome (MDS); both received long-term G-CSF and had partial response after two courses of IST. Fifteen percent of survivors had significant hypertension which persisted after CS was discontinued. This study shows promising response in children with AA treated with IST; however, the outcome was inferior to our institutional results with hematopoietic stem cell transplantation from a sibling donor. Hypertension and MDS are late complications. Longer follow-up, larger cohorts, and prospective studies are warranted to evaluate late complications and risk factors.
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