IgE Plasma Cell Leukemia Harboring t(11;14) and 1q Amplification.

IgE plasma cell neoplasm is the rarest subtype of plasma cell neoplasms and is known for its poor prognosis and high incidence of t(11;14). However, t(11;14) has been classified as a standard-risk rather than high-risk cytogenetic abnormality in multiple myeloma. We have been unable to explain the discrepancy that the hallmark of IgE plasma cell neoplasm with a poor prognosis is a standard-risk cytogenetic abnormality. Here, we report a case of IgE primary plasma cell leukemia with extramedullary lesions of the liver, stomach, and lymph nodes. Plasma cell infiltration was pathologically confirmed in each organ. Cytogenetic analysis of plasma cells revealed t(11;14) and amplification of 1q21. Chemotherapy, with immunomodulatory imide drugs, proteasome inhibitors, and CD38 antibodies, was unsuccessful. In IgE plasma cell neoplasm, coexistence of other cytogenetic abnormalities with t(11;14) may be important. Investigating the presence of cytogenetic abnormalities coexisting with t(11;14) is not only useful for evaluating prognosis but also important for understanding the pathogenesis of the disease. Recently, venetoclax, an oral BCL2 inhibitor, has demonstrated promising efficacy in plasma cell neoplasm patients harboring t(11;14). Development of an effective venetoclax-based regimen for treating aggressive IgE plasma cell neoplasm with t(11;14) is expected.

Multilineage Lymphoblastic Lymphoma as an Initial Presentation of Mixed Phenotype Acute Leukemia.

Mixed phenotype acute leukemia (MPAL) is characterized by leukemic blasts that express markers of multiple lineages. Compared with acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL), MPAL is considered to have a poor treatment outcome. We report a case of MPAL T/myeloid not otherwise specified that was initially presented as multilineage lymphoblastic lymphoma and subsequently developed into leukemic MPAL. An acute lymphoblastic leukemia-based treatment regimen was ineffective, but azacitidine and venetoclax therapy resulted in hematological complete remission. Our case suggests that multilineage lymphoblastic lymphoma should be considered to be the same disease as MPAL, albeit with different clinical presentations. Optimal treatment for MPAL has not been established yet, but azacitidine and venetoclax therapy may be a potential approach.

[Bilateral Multiple Liver Metastases after Pancreatoduodenectomy for a Duodenal Neuroendocrine Tumor-A Case Report].

The patient was a 79-year-old woman. In January 20XX, upper gastrointestinal endoscopy revealed a duodenal tumor with bleeding and ulceration. This tumor was diagnosed as a duodenal neuroendocrine tumor(NET)based on biopsy findings. In March 20XX, the patient underwent pancreatoduodenectomy with lymph node dissemination. Based on these pathological findings, the tumor was diagnosed as a duodenal NET(G2)with a lymph node metastasis(T2, N1, M0, Stage Ⅲ). Twenty months after the operation, abdominal CT revealed multiple liver metastases(S4, S7, and S8). After this recurrence, she underwent the subcutaneous somatostatin analogue injection therapy every 28 days, and transarterial chemoembolization( TACE)when these recurrent tumors showed remarkable regrowth, once a year, accounting for her age. She has maintained good disease control for 5 years.

N-Methyl-N-Nitrosourea-Induced Photoreceptor Degeneration Is Inhibited by Nicotinamide via the Blockade of Upstream Events before the Phosphorylation of Signalling Proteins.

N-methyl-N-nitrosourea (MNU), a known carcinogen, is generally used in animal models to chemically induce photoreceptor degeneration. It has been reported that nicotinamide (NAM) exerts a protective effect on MNU-induced photoreceptor degeneration. We investigated the molecular mechanisms on MNU-induced photoreceptor degeneration. Intraperitoneal MNU injection (75 mg/kg) in rats induced selective photoreceptor degeneration in 7 days. NAM administration completely inhibited photoreceptor degeneration. Photoreceptor layer abnormality was observed within 6 hours after MNU injection, whereas it was restored in the NAM-treated retina, as detected by optical coherence tomography. One day following MNU administration, phosphorylation of the cell death-associated signalling proteins c-Jun N-terminal kinase (JNK) and p38 mitogen-activated protein kinase (p38) increased, while the apoptosis-related proteins, full-length poly(ADP-ribose) polymerase (PARP) and apoptosis-inducing factor (AIF), were depleted. These changes were not observed in the NAM-treated retinas. Cell survival signalling, such as extracellular signal-regulated kinase (ERK), Akt, and cAMP response element binding protein (CREB) phosphorylation, increased in the MNU- but not in the NAM-treated rat retinas. Increased phosphorylated ERK (p-ERK) levels were observed within 6 hours after MNU administration, suggestive of cell survival signalling activation. This did not occur in NAM-treated retinas. These results indicate that NAM regulates upstream cellular events prior to the activation of cell death-related signalling events, such as JNK and p38 phosphorylation.

Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites.

POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. POEMS syndrome is a rare cause of refractory ascites. We report the case of a patient with POEMS syndrome presenting with massive ascites who was treated with very-low-dose lenalidomide and dexamethasone. A 57-year-old Japanese man was admitted to our hospital with pleural effusion, massive ascites, and leg edema. The diagnosis of POEMS syndrome was made based on the combination of the following findings: peripheral neuropathy, organomegaly, endocrinopathy, serum monoclonal protein elevation, skin changes, plasma VEGF elevation, and evidence of extravascular volume overload. Renal dysfunction induced by biopsy-proven renal involvement of POEMS syndrome was observed. Massive ascites of the patient dramatically diminished with long-time treatment of very-low-dose lenalidomide and dexamethasone. Lenalidomide seems to be a very promising therapy for POEMS syndrome presenting with extravascular volume overload such as edema, pleural effusion, and ascites. Very-low-dose lenalidomide might be effective especially for the patients with POEMS-related nephropathy.

Malignant peripheral nerve sheath tumor of the distal phalanx of the fifth toe: a case report.

Malignant peripheral nerve sheath tumor (MPNST) involving bone is rare. We report a case of MPNST of the fifth toe. The lesion was located in the distal phalanx of the right fifth toe and extended into surrounding subcutaneous tissues. Findings on magnetic resonance imaging and histological features of the case are described and the literature is briefly reviewed.

The natural degeneration course in the T1rho values of normal knee cartilage.

The purpose of our study is to investigate whether there is an age-related change in T1 rho values and to evaluate the effects of weight bearing on age-related increase in T1 rho values of normal cartilage. Thirty-two asymptomatic patients were examined using a 3.0T MRI to determine knee cartilage T1 rho values. Femorotibial and patella cartilage was defined as weight-bearing cartilage (WB-C) and non-weight-bearing cartilage (NWB-C), respectively. The femoral cartilage was divided into weight-bearing (WB-P) and less-weight-bearing (LWB-P) portions. Pearson's correlation coefficient and single regression analysis were used to assess the relationship between cartilage T1 rho values and age. The slopes of the regression lines of cartilage T1 rho values and age were compared between WB-C and NWB-C and between WB-P and LWB-P. Cartilage T1 rho values correlated positively with aging for all cartilage regions and all age groups (p<0.001). In the medial femoral cartilage, the age-related increase in T1 rho values was significantly greater for WB-P than for NWB-P (p<0.05). For several cartilage regions, this increase was greater for WB-C than for LWB-C (p<0.05). The T1 rho value is very sensitive to age-related cartilage degeneration and weight bearing-related degeneration, and hence may be a very sensitive and useful measure for the early diagnosis of osteoarthritis.

Magnetic resonance imaging (MRI) of articular cartilage of the knee using ultrashort echo time (uTE) sequences with spiral acquisition.

The objective of this study was to evaluate the sensitivity of ultrashort echo time (uTE) sequence for visualisation of calcified deep layers of articular cartilage. MRI with a uTE sequence was performed on five healthy volunteers. Signals from the calcified deep layers of the articular knee cartilage were evaluated on uTE subtraction images and computed tomography images. The calcified deep layers of the articular cartilage changed from having a low to a high signal when imaged with a uTE sequence. The reported uTE sequence was effective in imaging the deep layers of the knee cartilage.

A preliminary study of the T1rho values of normal knee cartilage using 3T-MRI.

INTRODUCTION: To investigate the degree of the effect of aging and weight-bearing on T1rho values in normal cartilage. MATERIALS AND METHODS: Thirty-two asymptomatic patients were examined using 3.0-T magnetic resonance imaging (MRI) to determine knee cartilage T1rho values and T2 values. The femoral and tibial cartilage was divided into weight-bearing (WB-Rs) and less-weight-bearing (LWB-Rs) regions. Single regression analysis was used to assess the relationship between cartilage T1rho values and age and between T2 values and age. Analysis of variance and post hoc-testing were used to evaluate differences in WB-Rs and LWB-Rs cartilage T1rho values and T2 values. Multiple linear regression modeling was performed to predict cartilage T1rho values. RESULTS: Cartilage T1rho values correlated positively with age for all cartilage regions tested (p<0.001). There were no significant correlations between cartilage T2 values and age. In both the medial femoral and tibial cartilage, T1rho values were significantly higher in WB-Rs than in LWB-Rs (p<0.05). There were no significant differences in T2 values between WB-Rs and LWB-Rs. Multiple linear regression analysis showed that both age and weight-bearing were significant predictors of increased medial knee cartilage T1rho values (p<0.001). CONCLUSIONS: Aging and the degree of weight-bearing correlate with the change in cartilage T1rho values. Based on multiple regression modeling, aging may be a more important factor than weight-bearing for cartilage T1rho values.

High-resolution MRI using a microscopy coil for the diagnosis of recurrent lateral patellar dislocation.

PURPOSE: Magnetic resonance imaging (MRI) has been commonly used for the preoperative evaluation of recurrent lateral patellar dislocation (RLPD). The purpose of this study was to determine the usefulness of high-resolution MRI (HR-MRI) with a microscopy coil for diagnosing RLPD. MATERIALS AND METHODS: The study group consisted of 15 patients with clinically diagnosed RLPD and 10 normal volunteers. All studies were performed on a 1.5-T MR system. First, conventional MRIs of the whole knee joint were obtained using the knee coil. Then HR-MRI scans using a microscopy coil in the medial aspect of the patella were obtained at the level of the superior pole of the patella, targeting the medial patellofemoral ligament (MPFL). The acquired HR-MRIs with RLPD were reviewed concerning the MPFL injury and the patellar injury. RESULTS: The MPFL was distinguished as a separate ligament, and the layer structure of the patellar cartilage was visualized clearly in all volunteers. The MPFL injury was visualized in 12 cases (87%); it included discontinuity, thickening, and loosening. The patellar injury was visualized in 11 cases (73%), which included dissecans of the medial margin and cartilage injuries. CONCLUSION: HR-MRI with a microscopy coil provides precise information of the MPFL and patellar cartilage injury for the diagnosis of RLPD.

Usefulness of MRI for diagnosis of painful snapping elbow.

Painful snapping of the elbows is rare. We report on a 12-year-old boy with a painful snap in both elbows. High-resolution magnetic resonance imaging of the elbow using microscopy coils detected a synovial fold interposed in each humeroradial joint and was very helpful in establishing the cause of symptoms. Resection of the synovial folds was performed with subsequent relief of symptoms.

Transcatheter embolization of splenic artery pseudo-aneurysm rupturing into colon after post-operative pancreatitis.

Splenic pseudoaneurysms following chronic pancreatitis can rarely become a source of life-threatening bleeding by rupturing into various regions or components, including pseudocysts, the abdominal cavity, the gastrointestinal tract, and the pancreatic duct. In such cases, prompt diagnosis and therapy are warranted. We report herein the case of a 52-year-old man in whom a splenic pseudoaneurysm ruptured into the colon via a fistula with an abscess cavity, causing massive bleeding, which was successfully managed by trans-catheter arterial embolization (TAE).

Use of the wallstent for symptomatic relief of malignant inferior vena cava obstructions.

PURPOSE: To report our experience with the Wallstent for the relief of congestive symptoms in patients with malignant inferior vena cava (IVC) obstructions. MATERIALS AND METHODS: We treated five patients with malignant obstructions of the IVC with a total of seven self-expandable stents. All obstructions were due to extrinsic compression; the procedures were indicated to relieve congestive symptoms in patients unresponsive to other therapies or in whom other treatments were contraindicated. Patients were evaluated for decrease in ascites and edema, weight loss, and patency, where available. RESULTS: All procedures were straightforward and obstructions could be successfully canalized. Inferior venacavograms obtained immediately after the procedure showed complete revascularization and disappearance of collateral circulation. Follow-up was carried out until death in four patients and until discharge in one. Of the five patients, four showed clinical improvement and one had no improvement. There was no recurrence of congestive symptoms. Except for one patient with partial migration of a stent, there were no important complications related to the procedure. CONCLUSION: Wallstents could be placed without complications in the IVC and were effective to ameliorate congestive symptoms in patients with malignant IVC obstructions.

Portosplenic blood flow separation in a patient with portosystemic encephalopathy and a spontaneous splenorenal shunt.

A patient with portosystemic encephalopathy, hyperammonemia, and a spontaneous splenorenal shunt was admitted to the authors' institution after a failed attempt at transvenous retrograde shunt obliteration. As an alternative approach, the authors separated splenic and portal flows by embolizing only the proximal splenic vein while leaving the shunt intact. Thus, the splenic flow could escape into the systemic circulation and an extreme increase in portal pressure was avoided. The procedure could provide rapid decreases in blood ammonia levels and a fast resolution of symptoms, but repeated interventions were required.