ABSTRACT
BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder with a variable clinical course. METHODS: A retrospective analysis was carried out of ITP patients presenting to a pediatric hematology-oncology department during a period of 20 years, with a focus on treatment and outcome. RESULTS: One hundred and twenty-four cases were recorded (mean patient age, 8.4 years). Forty-nine children (39.5%) had platelet counts <10,000/µL at diagnosis. No episode of severe bleeding was observed. Peak incidence was observed during spring and summer. Respiratory infections proceeded in 58% of cases. Treatment consisted of i.v. immunoglobulin (IVIG) in 93 children at four dosing schedules. Sixteen children received corticosteroids, 10 children received anti-D immunoglobulin and 14 received no treatment. Recovery was observed in 67% of children on IVIG and in 50% on anti-D globulin. Eight patients did not respond initially and received corticosteroids. Three children with refractory thrombocytopenia received anti-CD20 (rituximab). Fourteen children (11%) had persistent/chronic disease. In 10 of them recovery was observed in 13 months-8 years. Splenectomy was performed in six children with resistant/chronic disease. CONCLUSION: ITP has a benign course in the majority of cases. Anti-D globulin can effectively be used as an alternative first-line treatment. Rituximab can successfully be used in refractory cases, while splenectomy has currently limited indications.
Subject(s)
Purpura, Thrombocytopenic, Idiopathic , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Purpura, Thrombocytopenic, Idiopathic/drug therapy , Retrospective Studies , Time FactorsABSTRACT
The authors describe a 4-year-old girl with acute lymphoblastic leukemia in remission who developed fulminant hepatic failure due to varicella-zoster virus (VZV). Diagnosing VZV visceral infection in immunocompromised patients is often difficult due to atypical clinical presentation with few or no skin lesions and severe abdominal or back pain. Prompt initiation of empirical treatment with acyclovir and VZV immunoglobulin pending results of the serum polymerase chain reaction for VZV is warranted in this clinical setting.
Subject(s)
Chickenpox/virology , Hepatitis, Viral, Human/virology , Herpesvirus 3, Human/isolation & purification , Liver Failure, Acute/virology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/physiopathology , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Chickenpox/drug therapy , Child, Preschool , Fatal Outcome , Female , Hepatitis, Viral, Human/drug therapy , Humans , Immunocompromised Host , Liver Failure, Acute/drug therapy , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Remission InductionABSTRACT
Although the differentiation and classification of acute leukemia are based upon cytochemical features as well as immunologic, cytogenetic, and molecular characteristics, in many cases the morphological distinction of normal lymphocytes from lymphoblasts of acute lymphoblastic leukemia (ALL) is difficult using light microscopy. In this study the distinction between normal lymphocytes and lymphoblasts of childhood ALL is proposed using their spectral characteristics. The method has been based upon the analysis and classification of optical absorption characteristics of the bone marrow cells. Spectral microscopy system is capable of capturing a great number of narrow-band images, in the wide spectral range of the optical spectrum. The analysis showed statistically significant difference (P < 0.0001) between normal lymphocytes and lymphoblasts as far as it concerns the detection, identification and mapping of their spectral absorption characteristics. Our results suggest the potential of spectral imaging as a new method for the distinction of lymphocytes from lymphoblasts in cases that with the light microscope, the morphologic differences are not visible in the bone marrow smears at diagnosis or the follow up of the children with ALL.
Subject(s)
Lymphocytes/ultrastructure , Precursor Cell Lymphoblastic Leukemia-Lymphoma/physiopathology , Adolescent , Cell Nucleus/ultrastructure , Child , Child, Preschool , HumansABSTRACT
We investigated the characteristics of cord blood (CD) CD133(+) and CD34(+) cells, by flow cytometry, clonogenic assays and assessment of the replating ability (area under the curve (AUC)) following 7-day liquid culture in the presence of early acting growth factors and either thrombopoietin (TPO) or erythropoietin (EPO). The CD34(+) population showed a more effective proliferation in all parameters tested and TPO proved to be more effective than EPO. On the contrary, the CD133(+) cell fraction retained and expanded more immature elements in a modest but consistent manner with either TPO or EPO. We conclude that CD133(+) and CD34(+) expanded cord blood cells could potentially be used in combination to overcome the shortcomings of cord blood transplantation in older children and adults.
