[A case in which chromosome 5q deletion syndrome resistant to lenalidomide therapy transformed to refractory anemia with excess blasts].

An 80-year-old man was admitted to our hospital because of pancytopenia. Bone marrow examination revealed an increase in the number of dysplastic cells indicating trilineage dysplasia. A 5q13q31 deletion was the only genetic abnormality found, and consequently, 5q deletion syndrome was diagnosed. Although lenalidomide therapy was initiated, it had to be discontinued because of Stevens-Johnson syndrome, which occurred during the second course of treatment. There was no discernible hematological improvement, and bone marrow aspiration showed transformation to refractory anemia with excess blasts-2(RAEB-2)after lenalidomide therapy. However, by changing the therapy to azacitidine, cytogenetic remission was achieved.

[Clinicopathological features of intestinal complications in non-Hodgkin's lymphoma -a single institution analysis-].

We evaluated the clinicopathological features of patients who developed intestinal complications following surgery for gastrointestinal non-Hodgkin's lymphoma (NHL) and determined the risk factors for complications. We retrospectively analyzed 28 patients with gastrointestinal NHL who were treated at our institution between January 2007 and June 2012. Seven patients (25.0%) underwent surgery for bleeding, perforation, or ileus caused by the gastrointestinal NHL, particularly those with involvement of the jejunum or ileum. Half the patients with small intestinal NHL required surgery for complications; patients with this form of NHL were therefore considered to be at a high risk of complications. Those with semicircular ulcerative lesions, a protruding deformity, or systemic NHL involving the small intestine were also considered to be at a particularly high risk of intestinal complications.

[Bendamustine-rituximab therapy is effective for transformed follicular lymphoma with significant expression of p53].

We describe a patient with transformed follicular lymphoma(FL), expressing p53 but remaining in complete remission(CR) due to bendamustine-rituximab(BR)therapy. She was a 64-year-old female diagnosed with stage IV FL(grade 3A)in July 2007 when she was admitted with right lower abdominal pain and body weight loss. Colonoscopy revealed Bauhin' valve lymphoma of the terminal ileum, and computed tomography(CT)scan showed lymphadenopathy, involving the cervical, mediastinal para-aortic lymph nodes and right tonsil. She received chemotherapy with eight courses of CHOP therapy with rituximab and achieved CR. Two and a half years later, mediastinal lymph node swelling relapsed, and ibritumomab tiuxetan therapy induced the second CR. After ten months, however, a third relapse occurred as a submucosal tumor(SMT)of the stomach. Gastric SMT biopsy showed diffuse large B cell lymphoma(DLBCL)transformation with immunohistochemical expression of p53. Although gastric SMT disappeared after radiotherapy, which achieved the third CR, lymph node swelling was detected again in the para-aortic and-iliac artery lymph nodes in September 2011. Subsequently, she was treated with five courses of BR therapy, because bendamustine had been reported to be effective for p53 gene-deficient B cell neoplasms. The therapy was successful and achieved the fourth CR, demonstrating that BR therapy was effective for p53-expressing DLBCL.

[Acute promyelocytic leukemia presenting with central nervous system involvement at initial diagnosis].

We describe a rare case of acute promyelocytic leukemia (APL) presenting with central nervous system (CNS) involvement at the time of initial diagnosis. A 58-year-old male was hospitalized with palpitations, dyspnea, high grade fever, photophobia, and disturbance of consciousness in March 2010. APL was diagnosed by bone marrow (BM) examination. The cytogenetic analysis of BM cells demonstrated t(15;17)(q22;q11), and PML-RARA chimeric gene was detected by reverse transcriptase-polymerase chain reaction assay. Magnetic resonance imaging of the brain revealed several high intensity regions in the cerebrum and cerebellum. CNS involvement was diagnosed based on the appearance of APL blasts in cerebrospinal fluid (CSF). The patient was treated with all-trans retinoic acid (ATRA), and systemic chemotherapy consisting of idarubicin and cytarabine according to the Japan Adult Leukemia Study Group (JALSG) APL 204 protocol. He was then treated with continuous intrathecal administration of cytotoxic drugs (methotrexate, cytarabine, prednisolone) after systemic chemotherapy, achieving complete remission (CR) in both BM and the CNS. To date, he has been maintained in complete molecular remission in both BM and the CSF for 28 months, to date.

[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.

[A long term survivor of advanced gastric cancer treated with multi-drug combination chemotherapy].

We report a case of 70-year-old man who was admitted to our hospital due to hematemesis in June 2004. He was diagnosed by gastroscopy as having a type III moderately-poorly differentiated adenocarcinoma. A computed tomography (CT) scan revealed multiple lymph nodes swelling (#13, #16), finally he was diagnosed with gastric cancer stage IV (cT3, cN3, cM1). He was treated with S-1, but lymph nodes swelling increased in size, and then in March 2005, the treatment was changed to a second-line chemotherapy consisting of CPT-11 and CDDP. Abdominal CT scan showed a remarkable reduction of #16b1 lymph node, and the second-line chemotherapy was continued until 23 courses. But in April 2007, gastroscopy revealed the enlargement of gastric lesion. He was treated by third-line chemotherapy consisting of paclitaxel and doxifluoridine. This therapy was effective and continued until 7 courses. However, the treatment gradually became resistant and he died in May 2008, which was 4 years since the initial diagnosis.