High-grade B-cell lymphoma, not otherwise specified, presenting as primary peritoneal lymphomatosis and successfully treated with dose-adjusted EPOCH-R.

Peritoneal lymphomatosis (PL) is a rare lymphoma-associated condition defined as the dissemination of lymphoma cells in the peritoneum. An 82-year-old man presented with abdominal pain, heartburn, and high fever. Radiological findings, including positron emission tomography-computed tomography (PET-CT), and gastrointestinal fiberscopy, showed diffuse thickening of the peritoneum, omentum, and mesentery; however, no lymphadenopathy, hepatosplenomegaly, or gastrointestinal lesions were observed. Under suspicion of peritonitis carcinomatosa of unknown origin, exploratory laparoscopy was performed that revealed multiple white nodules and masses on the surfaces of the peritoneum, mesentery, and intestinal serosa. The histopathological and cytogenetic findings of the peritoneum revealed high-grade B-cell lymphoma, not otherwise specified, and a gain of MYC by fluorescence in-situ hybridization. The patient was treated with two cycles of R-CHOP therapy, followed by six cycles of dose-adjusted EPOCH-R therapy, and a complete metabolic response was confirmed by PET-CT. Since there are no specific radiological findings to confirm the diagnosis of PL, a histopathological diagnosis is usually required. Most PL exhibit an aggressive lymphoma phenotype and can be cured by appropriate chemotherapy. Therefore, early diagnosis and treatment are desirable.

Acute Abdomen and Adrenal Swelling as the First Manifestations of TAFRO Syndrome.

TAFRO syndrome, a rare systemic inflammatory disorder, commonly develops in an acute or subacute manner, with an aggressive clinical behavior. A substantial number of cases of TAFRO syndrome presenting with abdominal pain, and adrenal abnormalities on imaging have also been reported. A 54-year-old man developed severe acute abdominal pain. Bilateral adrenal swelling was detected on computed tomography. Although the abdominal pain resolved spontaneously, a fever and anasarca were observed. The patient was eventually diagnosed with TAFRO syndrome, and corticosteroid administration resulted in remission. TAFRO syndrome should be included in the differential diagnosis of acute abdomen and adrenal abnormalities.

Gastrointestinal Stromal Tumor in a Patient with Neurofibromatosis Type 1 That Was Successfully Treated with Regorafenib.

An unconscious 55-year-old man with a history of neurofibromatosis type 1 (NF1) was transported to the emergency department and was diagnosed with acute renal failure owing to a large bladder tumor. A submucosal tumor was also identified in the duodenum. The patient was diagnosed with a primary gastrointestinal stromal tumor (GIST) of the bladder and duodenum. After six cycles of regorafenib therapy, 18F-fluorodeoxyglucose accumulation in the duodenal GIST on positron emission tomography-computed tomography (PET-CT) showed a partial metabolic response. Currently, no standard drug therapy for unresectable or relapsed NF1-associated GIST has been established. Regorafenib may thus be considered as and appropriate initial therapy.

A new once-a-day fentanyl citrate patch (Fentos Tape) could be a new treatment option in patients with end-of-dose failure using a 72-h transdermal fentanyl matrix patch.

PURPOSE: The recommended dosing interval for transdermal fentanyl is every 72 h. However, some patients will have "end-of-dose failure," which may be seen as an increase of episodes of severe pain flares at the third day after application of the patch. A new once-a-day fentanyl patch was developed in Japan since 2010. This study aimed to assess the efficacy of the once-a-day fentanyl citrate patch for patients with cancer-related pain receiving the 72-h transdermal fentanyl not lasting 72 h. METHODS: We performed a cross-sectional retrospective analysis of 445 inpatients with the 72-h transdermal fentanyl at Higashi Sapporo Hospital. We could switch to the once-a-day fentanyl citrate patch if patients reported inadequate pain relief beyond 48 h after application of the 72-h transdermal fentanyl. Patients recorded baseline scores for background pain intensity (PI) and the frequency of use of daily rescue medication for breakthrough cancer pain (BTcP). RESULTS: Of all patients, 10.1% showed the increase in PI of 30% or more baseline PI on the third day after application of the 72-h transdermal fentanyl. Of patients, 84.4% were converted from equivalent dose of the 72-h transdermal fentanyl to the once-a-day fentanyl citrate patch. On the third day after switching, 60.5% of patients showed a reduction of more than 30% from baseline PI. Switching to the once-a-day fentanyl citrate patch significantly reduced the mean frequency of daily rescue dose for BTcP. CONCLUSIONS: A once-a-day fentanyl citrate patch provided stable pain control. Its use may be considered as the dominant strategy for patients receiving a 72-h transdermal fentanyl not lasting 72 h.

[Improvement of hepatomegaly after treatment with autologous hematopoietic stem cell transplantation followed by bortezomib and dexamethasone in Bence-Jones protein κ-type of multiple myeloma with systemic amyloidosis].

A 51-year-old man was admitted to our hospital complaining of right upper quadrant abdominal and back pain. Severe hepatomegaly (six fingerbreadths) was detected by liver palpation. Blood test results showed cholestatic liver disease. He was diagnosed with amyloidosis by liver biopsy. Bone marrow aspiration revealed 15% of contents to be plasma cells. BJPκ was detected by urine electrophoresis. Therefore, he was diagnosed with the BJPκ type of multiple myeloma with systemic amyloidosis. The patient was treated with bortezomib, dexamethasone and high-dose melphalan with autologous peripheral blood stem cell transplantation. He achieved VGPR (very good partial response) after transplantation. Hepatomegaly improved but swelling persisted, and he was therefore treated with 1.3 mg/m(2)/day of bortezomib and 20 mg/day of dexamethasone on day 1 and day 15 in 28-day cycles. Upon finishing 22 cycles in June 2014, his liver had returned to normal size. Restoration of normal liver size after treatment is rare in cases with severe hepatomegaly due to systemic amyloidosis. We thus report our case with a review of the relevant literature.

Safety and effectiveness of low-dose lenalidomide therapy for multiple myeloma complicated with bortezomib-associated interstitial pneumonia.

A 78-year-old woman was diagnosed with multiple myeloma (MM: IgG κ type, stage IIIA, ISS III) at a nearby hospital in August 2010. High-dose dexamethasone therapy was ineffective, and she was treated with 2 courses of bortezomib. She was referred to our hospital with back pain and dyspnea in November. She was diagnosed with interstitial pneumonia (IP) and improved rapidly with steroid pulse therapy. Because the involvement of bortezomib was suspected in IP, lenalidomide therapy was selected for MM. Lenalidomide (15 mg) was administered for 2 courses. The patient achieved a PR and the treatment is still ongoing with a good response. According to the interim report on PMS (post-marketing surveillance), 3 of the 1,177 patients treated with lenalidomide developed IP. The dose level was 25 mg in 2 cases and 10 mg in 1 case. The outcomes of these patients were death in 1 case, not recovered in 1 case, and unknown in 1 case. When lenalidomide is used to treat bortezomib-induced IP, there are no rules or regulations about its dose level. In the present case, the dose of lenalidomide (15 mg) was based on the retreatment dose after bone marrow suppression. Low-dose lenalidomide therapy was effective and safe against MM with a bortezomib-associated lung disorder.

[Systemic amyloidosis associated with IgD-λ multiple myeloma].

We describe here a case of systemic amyloidosis associated with IgD multiple myeloma. A 59-year-old man was admitted to our hospital in April 2009, because of macroglossia and swelling in both wrists and fingers. He had difficulty moving his limbs and was aware of peripheral neuropathy. Skin biopsy revealed extensive deposition of amyloidosis, which was positive by Congo red staining. Laboratory findings were as follows: serum electrophoresis revealed IgD λ monoclonal protein, and Bence-Jones protein was detected. Monoclonal IgD protein had a concentration of 727 mg/dl, and a bone marrow aspiration revealed 49.6% of plasma cells. These findings led to a diagnosis of IgD multiple myeloma with systemic amyloidosis. The patient was treated with MP (melphalan and methylprednisolone), high-dose dexamethasone and VAD therapy (vincristine, adriamycin and dexamethasone), but systemic amyloidosis progressed, and his general condition deteriorated. Coexistence of IgD multiple myeloma and systemic amyloidosis is rare, and accumulation of case reports is needed to gain a better understanding of this condition.

[Cyclic thrombocytopenia complicated with Sjögren syndrome and the mechanism of periodic platelet count fluctuation].

Cyclic thrombocytopenia is a rare disorder with periodic changes of the platelet count. We experienced a patient with cyclic thrombocytopenia and Sjögren syndrome (SS), and studied the mechanism of the cyclic changes of the platelet count. The patient, a 75-year-old woman, was referred to our hospital because of dry mouth, dry eyes, and severe thrombocytopenia. Her platelet count varied from 0.2 x 10(4)/microl to 22.7 x 10(4)/microl in 14-28-day cycles. Anti-SS-A/Ro antibody and Schirmer's test were positive. Histological examination of the salivary glands revealed infiltration of inflammatory cells, leading to the diagnosis of SS. There was an inverse relation between the platelet count and serum levels of PA-IgG. When the platelet count was low, the number of bone marrow megakaryocytes and the colony counts of CFU-Meg decreased. The serum thrombopoietin level increased at the nadir of the platelet count and decreased as platelets increased. After prednisolone therapy, her platelet count increased along with the improvement of sicca symptoms. These findings suggest that platelet fluctuation is due to the periodic increase of platelet destruction, as well as periodic failure of platelet production.