[Successful Subarachnoid Phenol Block Therapy in a Patient with Pelvic Tumor-related Rectal Tenesmus].

We report a successful case of subarachnoid phenol block therapy in a patient with refractory rectal tenesmus caused by metastatic breast cancer. A 50-year-old woman with pelvic metastasis of breast cancer had the constant desire for defecation and anal discomfort, although analgesics including opioids relieved her from low abdominal and anal pain. Computed tomography revealed rectal invasion of metastatic breast cancer and the diagnosis of tumor-related rectal tenesmus was made. Symptoms of rectal tenesmus were severe and drug-resistant. Subarachnoid phenol block in the sitting position, which was neurolytic saddle block and expected to inhibit rectal reflex, was performed and her symptom was dramatically relieved. The effect of the block continued until she died 19 days after. To our knowledge, we first report subarachnoid phenol block therapy for a patient with pelvic-tumor related rectal tenesmus.

[Efficacy of CT-guided splanchnic nerve block for persistent pain after fluoroscopy-guided nerve block in a patient with advanced pancreatic cancer].

We report a successful case of CT-guided splanchnic nerve block in a patient with advanced pancreatic cancer. A 76-year-old woman with epigastric distress was diagnosed with pancreatic cancer with multiple metastases. She underwent chemotherapy, but decided on best supportive care when her performance status worsened. Computed tomography revealed a 6 x 8 cm tumor mass in the pancreatic head. Oral oxycodone 20 mg x day(1) was ineffective and her Numerical Rating Scale (NRS) score was 9. Fluoroscopy-guided splanchnic nerve block with alcohol from L1-2 significantly reduced her pain, but she still required a fentanyl patch 2.1 mg x 3 days(-1) and loxoprofen (NRS 3). Four days later, we performed CT-guided splanchnic nerve block with alcohol 10 ml from T11-12. This significantly alleviated her pain and she was discharged uneventfully. She died 3 weeks after discharge. CT-guided splanchnic nerve block may be useful in cases of insufficient pain relief after fluoroscopy-guided splanchnic nerve block.

[Three successful cases of continuous dexmedetomidine infusion for the treatment of intractable delirium associated with cancer pain].

We report three cases of successful treatment of intractable delirium associated with cancer pain with continuous dexmedetomidine (DEX) infusion. Case 1 : An 83-year-old man receiving oral oxycodone for lung cancer pain developed delirium. He was resistant to haloperidol infusion, oral quetiapine, and opioid rotation. DEX infusion was administered at 0.4 microg kg-1 hr-1, and his delirium resolved. Case 2: A 50-year-old woman with cervical cancer of the uterus suffered from sepsis but could not take oral oxycodone. After continuous morphine infusion, she developed delirium. She was resistant to haloperidol infusion or injections of oxycodone for opioid rotation, but DEX infusion at 0.4 microg kg-1hr-1 led to disappearance of delirium symptoms. Case 3: A 71-year-old woman with advanced renal cancer was treated with epidural analgesia to alleviate cancer pain. She subsequently developed delirium but was resistant to haloperidol or chlorpromazine infusion. DEX infusion at 0.3 microg kg-1 hr-1 led to disappearance of delirium symptoms and orientation recovery. DEX infusion may be effective for the treatment of intractable delirium associated with cancer pain.

[Bone marrow-restricted involvement of T-cell granular lymphocyte leukemia].

A 66-year-old female was referred to our hospital with bone pain and progressive pancytopenia with granular lymphocyte infiltration only in the bone marrow (BM). Flow cytometric and histological analyses revealed that these cells were positive for CD3, TCRalphabeta, granzyme B, and the diagnosis of T-cell granular lymphocyte leukemia (T-GLL) with myelofibrosis was made. These BM granular lymphocytes were greatly ruffled and showed the CD3/CD20 double positive phenotype, which was not detected in the peripheral blood. The patient was treated with a single course of fludarabine followed by a favorable clinical course for 3 months. Many of the BM lymphocytes displayed almost normal appearance after treatment, however, the number of lymphocytes in the BM did not decrease and these were still CD3/CD20 double positive. This is an overlap case of T-GLL and peripheral T-cell lymphoma, unspecified (PTCLu).

Unbalanced translocation der(11)t(11;12)(q23;q13): a new recurrent cytogenetic aberration in myelodysplastic syndrome with a complex karyotype.

Cytogenetic abnormalities are observed in approximately one half of cases of myelodysplastic syndrome (MDS). Partial or complete chromosome losses and chromosome gains are frequently found, but there is a relatively high incidence of unbalanced translocations in MDS. We describe here two cases of MDS with an unbalanced translocation, der(11)t(11;12)(q23;q13). Both patients were 69 years of age and diagnosed with refractory anemia with excess of blasts in transformation (RAEB-t) according to the high percentage of blasts in the peripheral blood. Cytoplasmic hypogranulation of neutrophils was evident as a dysplastic change. The blasts were positive for CD4 and CD41a as well as CD13, CD33, CD34 and HLA-DR in both cases. Chromosome analysis showed complex karyotypes including a der(11)t(1;11)(q11;p15)t(11;12)(q23;q13) in case 1 and der(11)t(11;12)(q23;q13) in case 2 plus several marker chromosomes. Spectral karyotyping confirmed the der(11)t(11; 12)(q23;q13) and clarified the origin of marker chromosomes, resulting in del(5q) and del(7q). Fluorescence in situ hybridization (FISH) analyses with a probe for the MLL gene demonstrated that the breakpoints at 11q23 were telomeric to the MLL gene in both cases. FISH also showed that the breakpoint at 11p15 of the case 1 was telomeric to the NUP98 gene. Considering another reported case, our results indicate that the der(11)t(11;12)(q23;q13) is a recurrent cytogenetic abnormality and may be involved in the pathogenesis of advanced-stage MDS.

Philadelphia chromosome positive acute lymphoblastic leukemia showing normal karyotype in G-banding chromosomal examination before chemotherapy.

A 29-year-old male was admitted because of thrombocytopenia. A diagnosis of acute lymphoblastic leukaemia was made on the basis of a 61.6% infiltration of leukemic cells in his bone marrow. Standard G-binding chromosome analysis of bone marrow cells revealed a normal karyotype. He received combination chemotherapy, and achieved hematological complete remission. However, chromosomal analysis of bone marrow cells after 2 courses of consolidation therapy showed the Philadelphia (Ph) chromosome in two cells out of 20 analysed. We retrospectively examined the sample of bone marrow cells before chemotherapy; It showed minor BCR/ABL positivity with FISH and RT-PCR methods. The Ph chromosome disappeared after consolidation chemotherapy and allogeneic bone marrow transplantation, but the Ph chromosome reappeared at relapse. We postulated that there were two clones, both a Ph-positive clone and Ph-negative clone. At the initial diagnosis, Ph chromosome was not detected because the G-banding method analyzed only metaphase cells, which contained few Ph-positive clones. In order to offer effective therapy with molecular targeting agents, in this poor prognostic disease, it is necessary to detect Ph chromosome before the first chemotherapy and BCR/ABL detection with FISH or RT-PCR methods appears more useful than G-banding chromosome analysis.

The role of transcriptional coactivator TRAP220 in myelomonocytic differentiation.

The TRAP220 subunit of the thyroid hormone receptor-associated polypeptide transcription coactivator complex (TRAP/Mediator complex), mammalian counterpart of the yeast Mediator complex, is proposed to act on a variety of major and specific biological events through physical interactions with nuclear receptors. The vitamin D receptor (VDR) and retinoic acid receptor (RAR), coupled with retinoid X receptor (RXR), are nuclear receptors which have important roles for monopoiesis and granulopoiesis, respectively. In this study, we present the functional role of TRAP220 in nuclear receptor-mediated monopoiesis and granulopoiesis. The mouse Trap220(-/-) yolk sac hematopoietic progenitor cells were resistant to 1,25-dihydroxyvitamin D(3)-stimulated differentiation into monocytes/macrophages. Furthermore, flow cytometric analyses showed that HL-60 cells, human promyelocytic leukemia cell line, wherein TRAP220 was down-regulated, did not differentiate efficiently into monocytes and granulocytes by stimulation with 1,25-dihydroxyvitamin D(3) and all-trans retinoic acid, correspondingly. The expression of direct target genes of VDR or RAR, as well as the differentiation marker genes, was low in the knockdown cells. These results indicated a crucial role of TRAP220 in the optimal VDR- and RAR-mediated myelomonocytic differentiation processes in mammalian hematopoiesis.

Second case of CML with aberrant BCR-ABL fusion transcript (e8/a2) with insertion of an inverted ABL intron 1b sequence.

We found a case of Ph-positive chronic myelogenous leukemia (CML) patient with an atypical BCR-ABL transcript that was undetectable by a routine reverse transcription polymerase chain reaction (RT-PCR) for major BCR-ABL. Additional RT-PCR and sequence analyses have demonstrated that the aberrant transcript consists of a fusion of BCR exon 8 (e8) and ABL exon 2 (a2) with an insertion of a 55-bp inverted sequence of intron 1b between them. The nucleotide sequences of the aberrant transcript were identical to those of a previously reported CML patient. These are the only two CML cases in the literature with identical aberrant BCR-ABL transcripts.