Candida albicans endocarditis in a child with acute lymphoblastic leukemia: A dreaded complication of intensive chemotherapy.

Candida endocarditis is a rare entity during febrile neutropenia due to early introduction of empirical antifungal therapy. Early surgical intervention has diagnostic and therapeutic importance in Candida endocarditis. We report a case of Candida albicans endocarditis in a child with acute lymphoblastic leukemia on chemotherapy. The role of surgical intervention is discussed.

Infantile rhabdomyofibrosarcoma: A potentially underdiagnosed aggressive tumor.

Infantile Rhabdomyofibrosarcoma (IRMFS) is a rare clinicopathological entity that resembles infantile fibrosarcoma (IFS) but has ultrastructural and immunohistochemical evidence of rhabdomyoblastic differentiation. We report a 2 years and 6 months old boy who presented with a slowly progressive large soft-tissue mass in left axillary region. After complete excision, histopathology report revealed diagnosis of IFS. Review of the histopathology with immunohistochemistry (positive for desmin) revealed diagnosis of IRMFS. He was treated with aggressive adjuvant chemotherapy. He was in complete remission 6 months after completion of chemotherapy. In view of poor prognosis and aggressive treatment approaches for IRMFS, it must be differentiated from IFS to avoid under treatment.

Successful treatment of vincristine induced ptosis and polyneuropathy with pyridoxine and pyridostigmine in a child with acute lymphoblastic leukemia.

Vincristine is used in the treatment of solid tumors, lymphoma and leukemia in children. The dose-limiting toxicity is its neurotoxicity. We describe a 2-year-old girl with acute lymphoblastic leukemia who developed vincristine-induced polyneuropathy with bilateral ptosis and recovered on treatment with pyridoxine and pyridostigmine.

Significance of interleukin-6 (IL-6) and C-reactive protein (CRP) in children and young adults with febrile neutropenia during chemotherapy for cancer: a prospective study.

OBJECTIVES: We evaluated the usefulness of interleukin-6 (IL-6) and C-reactive protein (CRP) at the onset of febrile neutropenia and 72 hours later, in identifying risk groups and assessing response to antibiotic therapy. METHODS: All episodes of febrile neutropenia were divided in 3 study groups-microbiologically documented infection (MDI), clinically documented infection (CDI), and fever of unknown origin (FUO). Three outcome groups were defined as those responding to first-line antibiotics (R1), those responding to second-line antibiotics (R2), and those requiring antifungal therapy (RAF). Median values of IL-6 and CRP were compared between the groups. RESULTS: There were 57 episodes of febrile neutropenia among 26 patients younger than 25 years during 1 year of study period. On day 1, median IL-6 level was significantly lower in FUO group compared with CDI+MDI groups combined (P < 0.001). Rise in CRP on day 3 was highly significant to differentiate MDI group from other 2 groups (P < 0.001). The CRP also increased significantly on day 3 in RAF (P < 0.001) and R2 (P = 0.002) groups than in R1 group. CONCLUSIONS: Low level of IL-6 may help differentiate patients with FUO from those with documented infections. A rising CRP is indicative of serious infection.

Metronomic therapy for malignant peripheral nerve sheath tumor in neurofibromatosis type 1.

Malignant peripheral nerve sheath tumor (MPNST) is a highly aggressive tumor especially in the patients with neurofibromatosis type 1 (NF1). Without a complete surgical excision, prognosis is guarded. We describe a 10-year-old male with NF1 with MPNST, who had a local relapse within 5 weeks of surgical excision. Chemoradiotherapy did not result in tumor regression. Initiation of palliative oral metronomic therapy resulted in complete remission after six cycles. The patient continues to be in remission, 20 months after completion of nine cycles of metronomic therapy. Metronomic therapy may be effective in MPNST where conventional chemotherapy and radiotherapy fails.

Rituximab.

Rituximab is a chimeric mouse-human monoclonal antibody against the CD 20 antigen on the surface of B lymphocytes. It binds to CD20 and causes B cell death by antibody dependant cell-mediated cytotoxicity, complement mediated cytotoxicity and apoptosis. It leads to rapid and sustained depletion of B cells. It is licensed for use in adults with CD20 positive B-cell lymphoma and rheumatoid arthritis. In children, it has been used in a variety of off-label indications with promising results. It has proved useful as salvage therapy in relapsed refractory non-Hodgkins lymphoma and leukemia, and in hematological conditions including chronic immune thrombocytopenic purpura, hemophilia with inhibitors, and autoimmune hemolytic anemia. It has also proved effective in autoimmune conditions like primary systemic vasculitis and systemic lupus erythematosis. Nephrotic syndrome and opsoclonus-myoclonus syndrome are among the emerging indications for rituximab. In solid organ transplantation, rituximab is useful in the prevention and treatment of acute and chronic rejection as well as in post transplantation lymphoproliferative disease. Toxicity includes acute infusion reactions, susceptibility to bacterial infections, and reactivation of viral infections.

Isolated cerebellar involvement in posterior reversible encephalopathy syndrome in a child with acute lymphoblastic leukemia.

Parieto-occipital region is the most commonly involved site in posterior reversible encephalopathy syndrome (PRES). Cerebellar involvement has been reported with the predominant involvement of posterior cerebral regions, but isolated cerebellar involvement in PRES has been reported only once in English literature. We report here a 7-year-old boy with acute lymphoblastic leukemia who had PRES with isolated cerebellar involvement during induction chemotherapy. He presented with sudden onset headache, vomiting and hypertension followed by seizures, unconsciousness, and involuntary movements. Computed tomography scan revealed bilateral cerebellar hypodensities. He improved within few hours and reversibility of the lesions was documented on magnetic resonance imaging after 2 weeks. Awareness of atypical patterns in distribution of imaging abnormalities is important to recognize PRES more accurately and to avoid unnecessary diagnostic procedures and treatment.

Safe and efficacious use of procedural sedation and analgesia by non-anesthesiologists in a pediatric hematology-oncology unit.

BACKGROUND: Children often require relief of pain and anxiety while undergoing diagnostic and therapeutic procedures. Procedural sedation and analgesia (PSA) is the safe and effective control of pain, anxiety and motion so as to allow a necessary procedure to be performed and to provide an appropriate degree of memory loss or decreased awareness. OBJECTIVE: To prospectively describe procedural sedation and analgesia as performed in the pediatric oncology unit and to report the success of sedation and the incidence of complications. METHODS: IV Midazolam and IV Ketamine were used for PSA in pediatric oncology patients undergoing painful procedures. RESULTS: Between June 2004 and December 2004, 55 diagnostic and therapeutic procedures were performed using PSA in 16 children. There were 9 boys and 7 girls with a median age of 11 years. Twelve patients had hematolymphoid malignancies and 4 patients had solid tumors. The indication for PSA were bone marrow aspiration and or biopsy in 7 patients, therapeutic lumbar puncture in 43 patients, bone marrow aspiration and lumbar puncture in 4 patients and skin biopsy in 1 patient. All 55 procedures were successfully completed. Adverse events occurred in 15 (27%) episodes and included transient drop in oxygen saturation, vomiting, dizziness and disinhibition with crying spells. Average time to arousable state and full recovery was 22 minutes and 31 minutes respectively. None of the patients complained of post procedure pain nor recalled the procedure at the follow up visit. CONCLUSION: Procedural sedation and analgesia using midazolam and ketamine is a safe and efficient method of limiting anxiety and procedure related pain and can be successfully administered by non-anaesthesiologists. The complication rate is low and can be easily managed.

Blast crisis of chronic myeloid leukemia: diagnosis prompted by T(8;9).

T(8;9) is a relatively new translocation that has been reported in a few patients with chronic myeloid leukemia (CML) but never in acute myeloid leukemia (AML). We report here a patient who presented with AML with t(8; 9). He lacked the Philadelphia chromosome but tested positive for the gene by the polymerase chain reaction method. This confirmed the diagnosis of CML with blast crisis, and appropriate treatment could be instituted

Unrelated allogeneic bone marrow transplant in adrenoleukodystrophy using CD34+ stem cell selection.

Adrenoleukodystrophy is an inherited disease characterized by progressive neurodegeneration with rapid deterioration to a vegetative state. Of the treatment modalities tried to date, BMT is the only one with the potential for cure. However, the high rate of morbidity and mortality associated with allogeneic transplant makes it necessary to try out novel measures to improve the outcome in these patients. We report here a case where we used CD34+ stem cell selection for allogeneic unrelated-donor BMT in a patient with adrenoleukodystrophy.

Coexisting Gilbert's syndrome and sickle cell disease.

We report the coexistence of Gilbert's syndrome and homozygous sickle cell disease in a child with persistent unconjugated hyperbilirubinemia.