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The development of novel agents has markedly improved the prognosis of multiple myeloma(MM). However, salvage therapies for patients with MM that is refractory to novel agents and conventional chemotherapies have not been established. Herein, we describe successful treatments for such patients with the combination of clarithromycin, lenalidomide, and lowdose dexamethasone(BiRd)with or without melphalan and prednisolone(MP). Although its duration was relatively short, the remission is important in terms of the salvage strategy until the second generation of novel agents becomes available.

A five-month-old male infant with familial hemophagocytic lymphohistiocytosis underwent cord blood transplantation using reduced-intensity conditioning. Methylprednisolone (mPSL) pulse administration was performed for marked pulmonary edema during the early phase of transplantation, followed by GVHD treatment with mPSL until day 100. CMV antigenemia was detected on days 27 and 55, but serum became negative with 2- to 3-week ganciclovir (GCV) treatment on both occasions. On day 120, ophthalmological findings included multiple bilateral white spots and a positive PCR study using anterior chamber fluid confirmed the diagnosis of CMV retinitis affecting both eyes, although CMV antigenemia was negative. Re-treatment with GCV had a minimal effect on the ophthalmological findings, while foscarnet administration markedly improved the retinitis and decreased the CMV-DNA level. Considering that a substantial proportion of patients develop CMV retinitis even when CMV antigenemia is not present, routine monitoring involving ophthalmological examinations should be conducted for hematopoietic transplant patients, especially infants, who cannot complain of ocular symptoms.

The incidence of HHV-6 encephalitis during hematopoietic stem cell transplantation (HSCT) in children is thought to be less than that in adults and risk factors, prognosis and complications are virtually unknown. Herein, we report a pediatric case developing epileptic encephalopathy following HHV-6 encephalitis after a second cord blood transplantation (CBT). A 7-year-old boy with relapsed B-precursor acute lymphoblastic leukemia in second remission underwent CBT. However, he received a second CBT due to graft failure. On day 25 after the second CBT, he developed short-term memory defects and seizures. He was diagnosed with HHV-6 encephalitis because HHV-6 DNA was detected in his blood and cerebrospinal fluid and abnormal hippocampal signals were seen on cranial magnetic resonance imaging (MRI). After treatment with foscarnet, HHV-6 DNA levels and MRI findings improved; however, he developed epileptic encephalopathy five months after the onset of encephalitis. There are very few reports on pediatric epileptic encephalopathy associated with HHV-6 encephalitis after HSCT. Detailed studies are needed to analyze risk factors, prognosis, and complications.

A 49-year-old man was diagnosed with pure red cell aplasia (PRCA) based on low reticulocyte count (0.1%) and near absence of erythroblasts in the bone marrow (BM). While a small number of CD20-positive large abnormal cells was observed in the BM, Multiplex PCR confirmed B cell monoclonality. Gallium scintigraphy showed abnormality only in the BM, and we made a diagnosis of PRCA secondary to BM-derived B-cell malignant lymphoma. He was treated with rituximab-combined CHOP therapy with subsequent resolution of both disorders. He was further treated with high dose chemotherapy with auto-PBSCT, sustaining complete remission of the PRCA and lymphoma.

A 68-year-old female with palmoplantar pustulosis was referred to our hospital in July, 2009 because of liver dysfunction, a positive test for HTLV-1, and circulating abnormal lymphocytes with irregularly shaped nuclei. A diagnosis of acute type adult T cell leukemia/lymphoma (ATLL) was made based on generalized lymph node swelling and high levels of serum LDH, in addition to the findings described above. The associated palmoplantar pustulosis responded to some extent to antibiotics, steroid ointment, and narrow band UBV light irradiation. For ATLL, she was serially treated with CHOP chemotherapy, an LSG 15 protocol, and CytaBOM protocol with consequent partial remission. These chemotherapies did not affect the palmoplantar pustulosis. For ATLL in partial remission, we performed allogeneic peripheral blood stem cell transplantation (allo-PBSCT) from a related donor (HTLV-1-negative) with a conditioning regimen consisting of fludarabine, melphalan, and total body irradiation with 3 Gy in February, 2010. After the engraftment of donor hematopoietic cells, ATLL cells disappeared and the patient currently (as of April, 2012) remains in complete remission (CR). The residual palmoplantar pustulosis was further improved soon after allo-PBSCT and disappeared on Day 84 after transplantation. This refractory skin disease has also been in CR to date.

It has been reported that autoimmunity might be sometimes transferred from a donor to a recipient following allogenic bone marrow transplantation (allo-BMT). We report a patient to whom Basedow disease was transferred from the donor through an allo-BMT. A 18-year-old man with acute lymphoblastic leukemia, received the allo-BMT from his HLA-identical sister. Two-years later, he developed symptoms of palpitations and general fatigue. He was diagnosed as having Basedow disease because of hyperthyroidism, and high levels of the anti-thyroid stimulating hormone receptor antibody and antithyroid antibody. When he received the allo-BMT, his donor had neither the clinical symptoms of Basedow disease, nor abnormal findings on examination to determine her eligibility as a the donor. We retrospectively assayed anti-thyroid antibodies from their cryopreserved sera, and found the donor's anti-thyroid antibody was positive, while her serum was negative before transplantation. It was apparent that the donor had subclinical Basedow disease. The patient has remained in complete remission without any signs of chronic graft-versus-host disease (GVHD) up till the time of writing. It is believed that an anti-thyroid tissue reactive B-cell clone was transferred from the donor to the patient and commenced to produce antibodies. It is suggested that thorough investigation of the donor's autoimmunity is needed before allo-BMT. If the recipient develops an autoimmune disease after allo-BMT, we should definitely investigate the donor's autoimmunity.