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Case Report: Endothelial-targeted bridging therapy for a TTP-like phenotype in fulminant iMCD-TAFRO.

BACKGROUND: iMCD-TAFRO (the TAFRO clinical subtype of idiopathic multicentric Castleman disease (iMCD)) is characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Diagnosis is challenging due to its rarity, non-specific early presentation, and overlap with sepsis, lymphoma, and thrombotic microangiopathy (TMA). Endothelial injury is increasingly recognized as a central driver of its severe complications. CASE PRESENTATION: A previously healthy 20-year-old woman presented with rapid progression of fever, anasarca, jaundice, and respiratory failure. Laboratory

Waldenström Macroglobulinemia: Unusual Presentation With Cast Nephropathy/Light Chain Tubulopathy.

Cast Nephropathy/Light chain tubulopathy is usually present in patients with multiple myeloma and is very rare in patients with Waldenstrom Macroglobulinemia. There are very few case reports mentioned in the literature. We present an interesting case of Cast Nephropathy and light chain tubulopathy in an 81-year-old female patient with Waldenstrom Macroglobulinemia who required medical attention for worsening renal failure. Serum protein electrophoresis/Immunofixation showed IgM Kappa monoclonal gammopathy. Renal biopsy was remarkable for cast nephropathy and light chain tubulopathy. Furthermor

Clinical review: specific aspects of acute renal failure in cancer patients.

Acute renal failure (ARF) in cancer patients is a dreadful complication that causes substantial morbidity and mortality. Moreover, ARF may preclude optimal cancer treatment by requiring a decrease in chemotherapy dosage or by contraindicating potentially curative treatment. The pathways leading to ARF in cancer patients are common to the development of ARF in other conditions. However, ARF may also develop due to etiologies arising from cancer treatment, such as nephrotoxic chemotherapy agents or the disease itself, including post-renal obstruction, compression or infiltration, and metabolic o

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… Obstructive uropathy may nevertheless be present without hydronephrosis, during the first few days, when the collecting system is encased in retroperitoneal fibrosis or tumour or when the obstruction is partial [ 24 ]. … fibrosis, ureteral or bladder outlet obstruction) Table 2 Acute tumour lysis syndrome: associated malignancies, risk factors, clinical presentation and prophylactic treatment LDH, lactate dehydrogenase; TLS, tumour lysis syndrome. …

Acute Kidney Injury in the Patient with Cancer.

Over the last three decades, advancements in the diagnosis, treatment, and supportive care of patients with cancer have significantly improved their overall survival. However, these advancements have also led to a higher rate of cancer-related complications. Acute kidney injury (AKI) and chronic kidney disease (CKD) are highly prevalent in patients with cancer, and they are associated with an increased risk of all-cause mortality. This bidirectional interplay between cancer and kidney, termed "the kidney-cancer connection" has become a very active area of research. This review aims to provide

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… Retroperitoneal Fibrosis and Inapparent Obstructive Uropathy Radiology 1977 122 339 342 10.1148/122.2.339 834873 87. Curry N.S. Gobien R.P. Schabel S.I. Minimal-dilatation Obstructive Nephropathy Radiology 1982 143 531 534 10.1148/radiology.143.2.7071359 7071359 88. Spital A. Valvo J.R. …

Infiltrative and inflammatory systemic disorders involving the pituitary gland.

The hypothalamic-pituitary region can be affected by a wide and heterogeneous group of infiltrative and inflammatory disorders, including granulomatous, histiocytic, storage, infectious, non-pituitary neoplastic, and autoimmune or inflammatory conditions collectively grouped as hypophysitis. Although individually rare, these disorders are increasingly recognized due to growing awareness, wider use of pituitary imaging, and the emergence of immune checkpoint inhibitor-induced hypophysitis. They typically present with hypopituitarism (often including arginine vasopressin deficiency, which, parti

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… Typical manifestations include bilateral osteosclerotic lesions of the long bones, circumferential aortic wall thickening, and retroperitoneal fibrosis. …

Coexistence of Autoantibodies against the Golgi Complex and Ro52 Antigen in a Patient with Nonspecific Interstitial Pneumonia.

Nonspecific interstitial pneumonia (NSIP) is often associated with connective tissue diseases (CTD). The diagnosis of NSIP was confirmed in a 63-year-old man by high-resolution computed tomography and an open lung biopsy. Anti-Golgi complex autoantibodies (AGA) and anti-Ro52 antibodies were simultaneously detected at high concentrations. Autoantibodies to aminoacyl-tRNA synthetases (ARS) were negative. The patient was treated with corticosteroids for six months. During the seven-year follow-up, NSIP had a slow progression and patient had not developed the clinical features of CTD. The present

Idiopathic Multicentric Castleman Disease with Cutaneous Manifestation: Case Report.

Castleman disease constitutes a rare class of lymphoproliferative disorders, with an estimated incidence of 21 to 25 per million patient years. The idiopathic subtype exhibits a significantly diverse clinical presentation, which can imitate many autoimmune, malignant, and infectious diseases. Cutaneous manifestations are uncommon and require in-depth investigation, especially when concurrent lymphadenopathy is present. A 79-year-old female, with a chronic, complicated erysipelas-like lesion, presented with bilaterally enlarged inguinal lymph nodes; after surgical excision, their histopathologi

TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis.

TEMPI (telangiectasias, elevated erythropoietin level and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting) syndrome is a rare and newly defined multisystemic disease, which belongs to "monoclonal gammopathy of clinical significances". Due to its rarity, the etiology, pathogenesis, and clinical features of this disease remain largely unknown. Owing to its hidden and diverse clinical manifestations, missed diagnosis and misdiagnosis are common. In recent years, as more patients (including three fatal cases) were identified, some special clinical

New insights into therapeutic strategies for the treatment of peritoneal fibrosis: learning from histochemical analyses of animal models.

Encapsulating peritoneal sclerosis (EPS) is a fatal complication that can occur in patients undergoing long-term peritoneal dialysis. It is characterized by bowel obstruction and marked sclerotic thickening of the peritoneal membrane. Although the mechanisms underlying the development of EPS are complex, angiogenesis, inflammation, and peritoneal fibrosis are known to be essential factors. Now, several animal models that exhibit EPS have pathophysiology similar to that of human EPS and have been proposed for use in research to provide insights into it. Recent histochemical methods also help us

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… The synthetic estrogen tamoxifen has been used successfully to treat retroperitoneal fibrosis, a disease featuring the proliferation of fibrous tissue in the retro­peritoneum, and EPS [ 34 ]. …

A Rare Case of Aggressive Systemic Mastocytosis With Skeletal Osteosclerotic Lesions on Presentation: A Diagnostic Conundrum.

Systemic mastocytosis (SM) is a heterogeneous group of disorders caused by mast cell proliferation. SM often presents with non-specific symptoms making it a diagnostic challenge. Moreover, presentation with bone involvement is highly uncommon. Here, we report a rare case of SM in a 68-year-old female who initially presented with gastrointestinal symptoms and was later found to have sclerotic bone lesions on imaging. This case highlights an unusual presentation of SM, informing clinicians of the importance of keeping this disease process on the differential list of diagnostic conundrums.

Surgical approaches to fertility preservation in the cancer patient: current and future directions.

Advances in oncologic therapies have markedly improved survival among reproductive-age female patients, making fertility preservation an essential component of comprehensive cancer care. Gonadotoxic treatments, including chemotherapy, radiotherapy, and surgery, can disrupt ovarian and uterine endocrine function, resulting in premature ovarian insufficiency and infertility. This narrative review summarizes current and emerging surgical approaches to fertility preservation, organized by anatomic focus on the ovary, uterus, and cervix. Ovarian transposition preserves ovarian endocrine function by

The Proceedings of the 19th International Cancer Imaging Society Meeting and Annual Teaching Course : Verona, Italy. 7 - 9 October 2019.

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… Retroperitoneal fibrosis: Seen usually as an ill-defined sheet-like region of abnormality surrounding the infra-renal abdominal aort and IVC, which can encase and obstruct the ureters. Rarely it can be “mass-like” and mimick a primary retroperitoneal malignancy [Ref.8]. References 1. … Retroperitoneal fibrosis: typical and atypical manifestations. Br J Radiol 2000;73(866):214–22. A15 Differential Diagnosis of Renal Tumours H. …

Idiopathic multicentric Castleman disease treated with siltuximab for 15 years: a case report.

UNLABELLED: Human herpes virus-8 (HHV8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder sustained by pro-inflammatory cytokines, including interleukin-6 (IL-6). According to the international evidence-based criteria developed by the Castleman Disease Collaborative Network (CDCN), siltuximab, which works by inhibiting IL-6, is the recommended choice for iMCD treatment. We report a case of a 63-year-old white male with iMCD who has been on maintenance therapy with siltuximab for 15 years - representing one of the longest treatment periods of any

Cancer and the kidney: dangereoux liasons or price paid for the progress in medicine?

A long time ago, the links between renal disease and malignancy were observed, however, quite recently, their importance was recognized and 'new' subspecialty in nephrology, namely 'onconephrology' was established. In the XXI century, patients with malignancy make up the most growing number of the subjects seen for nephrology consult and/or critical care nephrology services. A plethora of renal problems may be found in patients with malignancy. They may influence not only their short-term outcomes but also the adequate therapy of the underlying oncological problem. Thus, all these kidney-relat

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… Ureteral obstruction due to retroperitoneal fibrosis can be also secondary to malignancy. Patients with cancer may also develop urinary tract obstruction that is unrelated to the malignancy (eg, benign prostatic hypertrophy in men). … However, in a case of obstructive AKI due to retroperitoneal fibrosis, malignancy or its treatment, hydronephrosis or hydroureter may not be present. It should be also stressed that radiotherapy of the pelvis or abdomen may also lead to retroperitoneal fibrosis. …

Role of Cardio-Oncology Rehabilitation in Hematopoietic Stem Cell Transplantation and Chimeric Antigen Receptor T-Cell (CAR-T) Therapy.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) and chimeric antigen receptor T-cell (CAR-T) therapy often lead to severe sarcopenia and cachexia during treatment, making it difficult to maintain exercise tolerance. Consequently, "cancer rehabilitation" programs have been implemented to sustain and improve physical activity and motor function. Hematologic malignancies often involve the use of cardiotoxic drugs. Moreover, graft-vs.-host disease associated with allo-HSCT and the cytokine release syndrome in CAR-T therapy elevate the risk of cardiovascular complications. Thus, esta

Резюме: 32 Второй Конгресс ЭСП и Международный конгресс XXXIII МПД.

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Extreme Peripheral Blood Plasmacytosis Mimicking Plasma Cell Leukemia as a Presenting Feature of Angioimmunoblastic T-Cell Lymphoma (AITL).

Angioimmunoblastic T-cell lymphoma (AITL) is one of four major subtypes of nodal peripheral T cell lymphoma, characterized by its cell of origin, the follicular helper T-cell (TFH). Patients typically present with prominent constitutional (B) symptoms, generalized lymphadenopathy, hepatosplenomegaly, cytopenias, and rash. Here we present a case of a 62-year-old male with progressive cervical adenopathy, fevers and weight loss presenting with extreme polyclonal plasmacytosis and high plasma EBV viral load. While the initial presentation appeared to mimic plasma cell leukemia or severe infection

61st Annual Conference of Indian Society of Gastroenterology, Virtual Diamond Jubilee ISGCON 2020, 19-20th December 2020.

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Ascites Index - an attempt to objectify the assessment of ascites.

IntroductionAscites is observed in cancer patients as well as in other non-neoplastic processes. In some patients, it may cause severe symptoms that can become directly life-threatening. The assessment of the degree of ascites seems useful in the determination of treatment effects as well as in the monitoring of fluid accumulation and early planning of decompression procedures.AimDetermination of the clinical usefulness of a quantitative method of determining the degree of ascites, so-called Ascites Index.Material and methodsThe Ascites Index is an ultrasonographic way of assessing the grade o

2021 Scientific Session of the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES), Las Vegas, Nevada, 31 August–3 September 2021: Video Loops

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