Shinji Kounami

Helicobacter pylori infection in children with chronic idiopathic thrombocytopenic purpura.

Abstract Background : Recently a high prevalence of Helicobacter pylori infection has been reported in adult patients with chronic idiopathic thrombocytopenic purpura (cITP). Furthermore, after H. pylori eradication therapy in such patients, their platelet counts have been observed to increase, suggesting that H. pylori may be a causative agent of adult cITP. However, there have been only a few reports of children with cITP. The purpose of the present paper was to examine the association between H. pylori infection and cITP in Japanese children.

Macrophage Activation Syndrome in Children with Systemic-Onset Juvenile Chronic Arthritis

Macrophage activation syndrome (MAS) is a life-threatening complication in children with rheumatic diseases, particularly systemic-onset juvenile chronic arthritis (SOJCA). Because of the potential fatality of this condition, prompt recognition and immediate therapeutic intervention are important. This study assessed the clinical features of nine MAS events in five children with SOJCA. Nonremitting fever and decreased platelet and white blood cell counts led to a diagnosis of MAS. The urinary β2-microglobulin (β2MG) level was a sensitive indicator of MAS. Serum levels of β2MG and soluble interleukin-2 receptor were also elevated. These biologic markers reflecting hyperactivated cellular immunity are useful indicators of MAS. Four children treated with cyclosporin A (CSP) achieved rapid and complete recovery, but one patient without CSP died due to rapidly progressive respiratory failure. All children treated with CSP responded quickly, and fever abated within 36 h of initiation of treatment. CSP should be added to first-line therapy of MAS.

Clinical assessment of Mycoplasma pneumoniae‐associated hemophagocytic lymphohistiocytosis

Background: Mycoplasma pneumoniae has been reported to be an etiologic pathogen of infection‐associated hemophagocytic lymphohistiocytosis (HLH), but few case reports have been available to date.

Additional Chromosome Abnormalities in Transient Abnormal Myelopoiesis in Down’s Syndrome Patients

We report a case of transient abnormal myelopoiesis (TAM) with Downs syndrome. As the blast cells had additional chromosome abnormalities (50,XY,+12,+14,+21 x 2), a possibility of acute leukemia was considered from a cytogenetic point of view. But the abnormal clone gradually disappeared without antileukemic therapies. Viewing it together with previous reports of TAM with additional chromosome abnormalities in Downs syndrome babies, its presence does not immediately indicate an aggressive clinical course as in acute leukemia and spontaneous complete hematological remission may be achieved. Thus, a careful follow-up should precede cytogenetic findings helping to determine the therapeutic intervention.

Primary anaplastic large cell lymphoma of the psoas muscle: a case report and literature review.

Primary anaplastic large cell lymphoma (ALCL) of skeletal muscle is very rare. We report a case of ALCL arising from the left psoas muscle. A 14-year-old girl presented with a large left inguinal tumor. She complained of a 2-month history of left leg pain, which had been exacerbated upon leg extension, and she had become aware of a rapidly growing left inguinal tumor 3 weeks before admission. CT scan and MRI revealed a large tumor arising from the left major psoas muscle and protruding into the inguinal region. In view of the tumor’s location and the patient’s age, soft tissue tumors such as rhabdomyosarcoma and primitive neuroectodermal tumor were initially considered. However, histopathological examination yielded a diagnosis of anaplastic lymphoma kinase-positive ALCL. The serum level of soluble interleukin-2 receptor was markedly elevated at 50,414 U/ml, and this also strongly suggested ALCL. Although rarely reported, ALCL is an important entity to consider in the differential diagnosis of skeletal muscle tumors in children and young adults.

Myelodysplastic syndrome after regression of transient abnormal myelopoiesis in a Down syndrome infant : Different clonal origin?

A boy with Down syndrome who developed myelodysplastic syndrome after regression of transient abnormal myelopoiesis (TAM) is described. His blast cells in TAM had other chromosome abnormalities in addition to trisomy 21;50,XY,+21c,+12,+14,+21. Serial chromosome analysis in follow-up showed abnormal clones involving monosomy 7. Myelodysplastic syndrome was diagnosed. Because two clones had different karyotypes, they might have derived from different clones.

Severe Hypercalcemia in a Child with Acute Nonlymphocytic Leukemia: The Role of Parathyroid Hormone-Related Protein and Proinflammatory Cytokines

Among the hematological malignancies, hypercalcemia has often been reported in lymphoid malignancies such as multiple myeloma and adult T cell leukemia/lymphoma, but it has only rarely been described in acute nonlymphocytic leukemia. We describe here a 14-month-old girl with acute monocytic leukemia complicated by severe hypercalcemia (4.6 mmol/l) at presentation. A bone survey showed generalized bone resorption, but no localized osteolytic lesions. A search for the etiology of the hypercalcemia revealed that the serum levels of parathyroid hormone-related protein (PTHrP) and also proinflammatory cytokines with stimulatory effects on osteolytic bone resorption – TNF-α, IL-6 and M-CSF – were elevated. The patient achieved complete remission with induction chemotherapy, and the levels of PTHrP and the cytokines became normalized. In this case, PTHrP and cytokines might have acted cooperatively to exacerbate bone resorption, resulting in severe hypercalcemia.

Olfactory neuroblastoma as a second malignant neoplasm in a patient previously treated for childhood acute leukemia

Various kinds of second malignant neoplasms after successful treatment for childhood acute leukemia have been reported. The authors describe an unusual case of an olfactory neuroblastoma in a patient previously treated for childhood acute leukemia including autologous bone marrow transplantation. The prophylactic cranial irradiation and the total body irradiation during autologous bone marrow transplantation may have induced the development of their patients olfactory neuroblastoma. Although a second primary olfactory neuroblastoma is rare, it should be added to the list of second malignant neoplasmsin the sinonasal region.

Early-Onset Hemophagocytic Lymphohistiocytosis after the Start of Chemotherapy for Advanced Neuroblastoma

The authors report the clinical course of a 3-year-old boy with stage 4 neuroblastoma (NB) complicated by hemophagocytic lymphohistiocytosis (HLH) immediately after the start of chemotherapy. The NB responded very well to the chemotherapy, but the patient developed high fever on the 2nd day, and was diagnosed as having HLH of the 7th day of chemotherapy. No infections were demonstrated, and massive tumor cell destruction resulting from the rapid effect of chemotherapy was thought to be a cause of systemic cytokine response, resulting in HLH. Methylprednisolone pulse therapy was effective for the HLH, which did not recur thereafter. HLH should be recognized as a serious adverse event during chemotherapy for advanced NB that has a large malignant cell load.

Non-myeloablative allogenic peripheral blood stem cell transplantation in a patient with refractory osteosarcoma

Abstract:  The prognosis of patients with relapsed osteosarcoma is dismal despite the use of intensive chemotherapy. We describe a patient with refractory osteosarcoma who underwent non‐myeloablative peripheral blood stem cell transplantation (PBSCT) from an human leukocyte antigen (HLA)‐identical sibling during a third complete remission. The patient suffered pulmonary relapse after the transplantation. Cyclosporin A withdrawal induced a graft‐vs.‐osteosarcoma effect and graft‐vs.‐host disease, but eventually the tumor progressed. Although our experience in this case suggested the presence of a graft‐vs.‐osteosarcoma effect during non‐myeloablative allogenic PBSCT, this strategy might have limited value for refractory osteosarcoma with rapid growth kinetics.