Complete Remission in a TEMPI Syndrome Treated with a Daratumumab, Lenalidomide, and Dexamethasone-Based Regimen: A Case Report.

Introduction: TEMPI syndrome is a rare and acquired condition which is characterized by five classical features: telangiectasias, erythrocytosis with elevated erythropoietin, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting. The classical treatment is based on bortezomib which can achieve variable responses. Relapse or refractory disease may occur, so other treatment strategies can be proposed. Case Presentation: We describe the case of a 54-year-old male followed for a refractory TEMPI syndrome who achieved complete remission after a second-line therapy composed of daratumumab-, lenalidomide-, and dexamethasone-based regimen (DLd). He achieved a complete remission with dramatic improvement of his renal function, restitution of a normal blood oxygen, and disappearance of polycythemia. Conclusion: This case highlights the effectiveness of an association of DLd to treat refractory TEMPI syndrome. We also provide arguments for an association between TEMPI syndrome and monoclonal gammopathy of renal significance.

Extensive Cervico-Thoraco-Abdominal Attacks of Angioedema: CT Diagnosis in Two Unusual Cases.

Angioedema (AE) classically manifests as an acute transient swelling of extra-visceral spaces, subcutaneous and submucosal tissues. Sometimes it may be a life-threatening condition. The causes are numerous, and the common denominator is an increased vascular permeability allowing diffusion or extravasation of fluid from the vascular bed to the interstitial space. The severity of AE is related to the cause, body location, and extension. We hereby report two very unusual cases characterized by a massive attack of AE from the left cervical area to the pelvis through the length of the mediastinum and axial posterior retroperitoneum. The diagnosis was established by CT. The first case was found related to drug intake, and the second appeared idiopathic.

The clinical relevance of imatinib plasma trough concentrations in chronic myeloid leukemia. A Belgian study.

This retrospective multicenter study in patients with chronic myeloid leukemia in chronic phase was undertaken to confirm the clinical relevance of imatinib plasma concentrations monitoring in daily practice. Forty-one patients, with 47 imatinib plasma measurements, were analyzed during treatment with imatinib given at a fixed 400mg daily dose. A significant inverse relationship of imatinib concentration with the patients' weight was observed (Pearson's test: p=0.02, R2=0.1). More interestingly, patients with poor response (switched to another tyrosine kinase inhibitor because of imatinib failure, or because of disease progression after an initial response) displayed a significantly lower mean imatinib concentration as compared to patients maintained on imatinib (822ng/mL vs 1099ng/mL; Student's t-test, p=0.04). Failure or disease progression occurred more often in patients in the lowest quartile of imatinib concentrations compared to patients in the highest quartile (p=0.02, logrank test). No correlation could be established with other biological or clinical parameter, including complete cytogenic response and major molecular response. IN CONCLUSION: in patients treated with imatinib at a fixed daily dose of 400mg, imatinib plasma concentrations decreased with increasing body weight and were lower in patients switched to another tyrosine kinase inhibitor due to imatinib failure. Systematic determination of imatinib plasma trough levels should be encouraged in such patients.

A Rare Case of Adult Acute Ileocecal Ischemia Related to Henoch-Schönlein Purpura: MDCT Findings.

Henoch-Schönlein purpura (HSP) is a form of immune complex-mediated leukocytoclastic vasculitis involving the skin and other organs. It primarily affects children. The occurrence of HSP in adults is rare, and gastrointestinal (GI) involvement is one of its most common clinical manifestations. The GI symptoms are caused by hemorrhage and edema within the bowel and wall mesentery. Complete recovery usually occurs, and life-threatening complications are rare. We report a typical case of GI involvement of the ileocecal area diagnosed with multidetector computed tomography (MDCT) and confirmed by skin biopsy.

Small bowel obstruction caused by peritoneal immunoglobulin g4-related disease mimicking carcinomatosis: case report.

We hereby report a case of diffuse pelvic peritoneal involvement by immunoglobulin G4-related disease (IgG4-RD). Numerous pelvic masses and nodules showing delayed enhancement on enhanced abdominal CT were found to congregate in the pelvic organs of a 57-year-old female presenting with intestinal subocclusion. The differentiation between peritoneal IgG4-RD and pelvic peritoneal carcinomatosis was only made by histopathology and immunohistochemistry performed after surgical resection. Autoimmune pancreatitis represents the historical prototype of IgG4-RD, but the spectrum of manifestations involving various organs has expanded during the last decade. In this report, we shortly review this clinical entity.

MDCT findings in primary amyloidosis of the greater omentum and mesentery: a case report.

Imaging findings of amyloid infiltration of the greater omentum, mesentery, and retroperitoneal spaces have only extremely rarely been reported in the radiological literature. This report illustrates the MDCT findings fortuitously found in a 70-year-old male presenting with a known latent myeloma.Extra abdominal deposits-axilla and cardiophrenic angles-were first fortuitously found during thoracic MDCT. Secondary abdominal MDCT revealed the extensive abdominal spread that consisted of very diffuse but asymptomatic pseudo carcinomatous hazy omental, mesenteric and-in a minder proportion-retroperitoneal deposits; these remained isolated without calcification, lymphadenopathy, ascites, or any sign of associated bowel wall thickening. A specific definite histologic diagnosis was made without laparotomy through a biopsy in the right axilla.