Ryohei Abe

Successful treatment of tafro syndrome with tocilizumab, prednisone, and cyclophosphamide

TAFRO syndrome is a rare systemic inflammatory disease characterized by thrombocytopenia, pleural effusion, fever, renal dysfunction, reticulin fibrosis of the bone marrow, and organomegaly. The clinical course varies significantly among patients. However, the prognosis is usually dismal in patients with severe TAFRO syndrome, and no optimal treatment has yet been established. We herein describe the first case of TAFRO syndrome, which was successfully treated with combination therapy consisting of tocilizumab, prednisone, and cyclophosphamide.

Successful treatment with bendamustine and rituximab for paraneoplastic pemphigus

Dear Editor, Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with neoplasms, particularly lymphoid malignancies [1, 2]. PNP is characterized by painful stomatitis, polymorphous skin eruption, and the presence of antibodies against desmogleins (Dsg) 1 and 3, envoplakin, and periplakin [1, 2]. Various treatments have been applied to PNP, including corticosteroid, cyclophosphamide, plasmapheresis, and rituximab, but their efficacy has been limited and an optimal treatment remains to be established. We herein report a case of follicular lymphoma developing PNP that was successfully treated with bendamustine and rituximab. A 74-year-old woman noticed rapidly progressing left axillary lymphadenopathy. She had a 23-year history of follicular lymphoma (histological grade 2) and had been receiving a series of treatments including CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone), MACOP-B (methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomicin), rituximab alone, and local radiation therapy. The adenopathy was histologically diagnosed as a recurrence of follicular lymphoma. The lesion was treated with local radiation therapy (total 36 Gy) delivered against the lesion. Two months after the initiation of radiation therapy, blisters developed at the radiation site (from the left shoulder to the back) along with eruptions and blisters at the oral, vaginal, and anal mucosa (Fig. 1a, b). Prednisolone (0.5 mg/kg/ day) was initiated but the lesions remained unchanged. The patient was referred to our hospital’s dermatology service. The histopathological studies of the skin and oral mucosa lesions showed suprabasal blister, acantholysis, and basal cell layer degeneration. Immunoblot analysis of the serum revealed the presence of antibodies against envoplakin, periplakin, Dsg1, and Dsg3. The anti-Dsg3 antibody value was 47.5 U/mL (normal range, less than 20.0 U/mL). On the basis of these findings, a diagnosis of PNP was made. Prednisolone was increased to 1.0 mg/kg/day, but there was no improvement. Bendamustine (90 mg/m on days 1, 2) in combination with rituximab (375 mg/m on day 1), BR, was initiated. After 2 weeks, the skin lesions and oral eruption had begun to improve and were almost resolved at 4 weeks (Fig. 1c, d). During the treatment, cytomegalovirus reactivation and neutropenia developed and were successfully treated with an antiviral agent and granulocyte colony-stimulating factor. After two courses of BR, anti-Dsg3 antibodywas undetectable. At 1 year after the initiation of BR treatment, prednisolone administration was successfully tapered to 6 mg/day without recurrence of PNP or follicular lymphoma. Although corticosteroid has been used for the treatment of PNP, it has not been effective for mucosal lesions of PNP [1–5]. Recently, the efficacy of rituximab against PNP associated with malignant lymphoma has been reported [6]. Bendamustine has been used as both a firstand second-line therapy for follicular lymphoma [7, 8]. Although there was a case of PNP developing after BR chemotherapy for follicular lymphoma [9], we observed that BR dramatically improved PNP lesions. The inconsistency between these findings suggests that the pathogenesis and clinical features of PNP associated with malignant lymphoma are diverse. * Taku Kikuchi taku_k_1123@mac.com

Recurrent bacterial pneumonia due to immunoglobulin G2 subclass deficiency after allogeneic hematopoietic stem cell transplantation: Efficacy of immunoglobulin replacement

Immunoglobulin (Ig) G2 subclass deficiency is known to be associated with recurrent bacterial respiratory infections caused by capsulated bacteria. We encountered a case of recurrent pneumonia due to Streptococcus pneumoniae after allogeneic hematopoietic stem cell transplantation (HSCT). IgG2 subclass level was specifically low, and prophylactic Ig replacement successfully prevented subsequent infections. However, the cessation of Ig replacement resulted in subsequent pneumonia. These findings suggested that IgG2 deficiency could be a cause of recurrent pneumococcal infection after allogeneic HSCT.

Invasive hepatic mucormycosis: A case report and review of the literature

Mucormycosis generally develops under immunocompromised conditions, including hematological malignancies and solid organ or hematopoietic stem cell transplantation. Although mucormycosis usually affects the lungs and paranasal sinuses, sporadic cases of invasive mucormycosis of the liver have been reported. We hereby report a patient with myelofibrosis who developed hepatic mucormycosis diagnosed by post-mortem examination. An extensive literature review identified 13 reported cases of hepatic mucormycosis, including ours, without lung involvement. Most of the underlying diseases or conditions were hematological malignancies and solid organ transplantation. Three cases had splenic lesions and four had gastrointestinal lesions, suggesting the possibility of translocation to the liver and/or spleen from the gastrointestinal tracts. Hepatic mucormycosis should be recognized as one of the presentations of invasive mucormycosis, especially when hepatic nodules are found in immunocompromised patients such as those with hematological malignancy or recipients of solid organ transplantation.

Long-term Eculizumab Treatment Contributes to Recovery from End-stage Renal Disease Caused by Atypical Hemolytic Uremic Syndrome

We experienced a favorable outcome in an adult case of atypical hemolytic uremic syndrome (aHUS) after long-term eculizumab treatment. A 38-year-old Japanese man with a history of central retinal vein occlusion was admitted to our hospital with progressive dyspnea. He was found to have non-immune hemolytic anemia, thrombocytopenia, and acute renal failure two weeks after an episode of the common cold. Plasma exchange was ineffective; therefore, we initiated eculizumab after we excluded other thrombotic microangiopathies. Although long-term peritoneal dialysis was required, we successfully discontinued dialysis 18 months after the onset of aHUS with eculizumab.