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Unicentric mixed variant Castleman disease associated with intrabronchial plasmacytoma.

UNLABELLED: Castleman disease (CD), described as a heterogeneous lymphoproliferative disorder, can be divided into different subtypes according to clinical appearance (unicentric and multicentric form) and histopathological features (hyaline vascular, plasma cell, mixed type, human herpesvirus 8-associated and multicentric not otherwise specified). Unicentric CD is known to be usually of the hyaline vascular variant, plasma cell and mixed type of this form are quite uncommon. Malignancies are mainly associated with the multicentric form. We report a rare case of unicentric mixed variant CD evo

Long term outcomes of pediatric liver transplantation according to age.

Liver transplantation (LT) has been the key therapy for end stage liver diseases. However, LT in infancy is still understudied. From 1992 to 2010, 152 children had undergone LT in Seoul National University Hospital. Operations were performed on 43 patients aged less than 12 months (Group A) and 109 patients aged over 12 months (Group B). The mean age of the recipients was 7 months in Group A and 74 months in Group B. The patients' survival rates and post-LT complications were analyzed. The mean Pediatric End-stage Liver Disease score was higher in Group A (21.8) than in Group B (13.4) (P = 0.0

Gene therapy for haemophagocytic lymphohistiocytosis.

Haemophagocytic lymphohistiocytosis (HLH) describes a severe and often fatal immunodysregulatory disorder caused primarily by the uncontrolled activation and proliferation of T cells and macrophages. A number of genetic defects mainly involving defective granule exocytosis and effector cell cytotoxicity have been identified and well characterised at the molecular and cellular level. These conditions have limited therapeutic options and given the predominant restriction of the causative gene to the haematopoietic system, they have become attractive targets for haematopoietic cell gene therapy.

Unicentric Castleman's Disease Arising from an Intrapulmonary Lymph Node.

Castleman's disease is an uncommon lymphoproliferative disorder of unknown etiology, most often involving the mediastinum. It has 2 distinct clinical forms: unicentric and multicentric. Unicentric Castleman's disease arising from an intrapulmonary lymph node is rare, and establishing a preoperative diagnosis of this disease is very difficult mainly due to a lack of specific imaging features. We report a case of intrapulmonary unicentric Castleman's disease in an asymptomatic 19-year-old male patient who was accurately diagnosed by preoperative computed tomography (CT). The mass was incidentall

A case of cervical multicentric Castleman disease treated with intensity-modulated radiation therapy using helical tomotherapy.

Castleman disease (CD) is a rare lymphoproliferative disorder. Two clinical entities are described: a unicentric form with disease confined to a single lymph node region and a multicentric form characterized by generalized lymphadenopathy and systemic symptoms. Although surgery is regarded as standard therapy for the unicentric form, no consensus has been reached concerning the standard treatment for multicentric CD. We report here a case of cervical multicentric CD treated with intensity-modulated radiation therapy (IMRT), using helical tomotherapy to minimize xerostomia in comparison with co

Disseminated histoplasmosis in a juvenile lupus erythematosus patient.

INTRODUCTION: Histoplasmosis is an infection caused by dimorphic fungus, Histoplasma capsulatum, and it has not been reported in juvenile systemic lupus erythematosus (JSLE) patients, particularly progressive disseminated histoplasmosis (PDH) subtype. CASE REPORT: We reported herein a 14-year old girl who was diagnosed with JSLE. Six months later, she had abdominal distension and received prednisone, hydroxychloroquine and azathioprine. Computer tomography evidenced hepatosplenomegaly and multiple mesenteric, mediastinal and retroperitoneal enlarged lymph nodes, forming large conglomerates at

The Schnitzler syndrome.

The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. It associates a chronic urticarial skin rash, corresponding from the clinico-pathological viewpoint to a neutrophilic urticarial dermatosis, a monoclonal IgM component and at least 2 of the following signs: fever, joint and/or bone pain, enlarged lymph nodes, spleen and/or liver, increased ESR, increased neutrophil count, abnormal bone imaging findings. It is a chronic disease with only one known case of spontaneous remission. Except of

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… About nineteen percent of the reported patients with this syndrome developed lymphoproliferative disorders [ 3 , 6 ], a percentage close to the 18% prevalence at 10 years of lymphoproliferative disorders in patients with IgM MGUS in general [ 1 ]. … In rare instances, treatment of the underlying lymphoproliferative disorder had a beneficial effect on the Schnitzler syndrome, supporting the former point of view [ 60 ]. …

Systemic Epstein-Barr virus infection associated with membranous nephropathy in children.

Epstein-Barr virus (EBV) infection can cause diverse renal manifestations ranging from microscopic hematuria to acute renal failure. Membranous nephropathy (MN) is an uncommon and usually secondary cause of nephrotic syndrome in children, and has been reported after chronic infections and antigenemia. We report two pediatric cases of secondary MN associated with acute and chronic systemic EBV infection. Patient 1 had a liver transplant for cirrhosis due to biliary atresia and developed chronic EB viremia. Membranous nephropathy occurred 3 years later and with aggressive therapy has partially s

Outcome and late complications of radiotherapy in patients with unicentric Castleman disease.

Castleman disease is a rare lymphoproliferative disorder. Surgery is considered standard therapy for the unicentric type. However, case reports have documented favorable responses to radiotherapy. The aim of this study was to analyse the clinical outcomes of five patients with unicentric Castleman disease treated with radiotherapy between 1991 and 2005. Mediastinal lymph nodes were the most common site of disease (four patients). Three patients were treated with radiotherapy alone, two patients with surgery and radiotherapy. Patients were treated with radiotherapy doses ranging from 40 Gy to 5

Causes and consequences of the autoimmune lymphoproliferative syndrome.

Autoimmune lymphoproliferative syndrome (ALPS) is the first autoimmune hematological disease whose genetic basis has been defined. It is a disorder of apoptosis in which the inability of lymphocytes to die leads to lymphadenopathy, hypersplenism, and autoimmune cytopenias of childhood onset. More than 200 ALPS patients have been studied over the last 15 years and followed by our colleagues and ourselves at the Clinical Center of the National Institutes of Health. Based upon this experience we have determined that patients with germline mutations of the intracellular domain of Fas protein, the

[Transitional type of Castleman's disease manifested as the POEMS syndrome].

BACKGROUND: Castleman's disease is an atypical lymphoproliferative disorder characterized by angiofollicular hyperplasia of lymph nodes. Histologically, it can be classified into a hyaline-vascular type, plasma-cell type, and transitional (mixed-cell) type, while clinically localized type has been classified as unicentric, or generalized (multicentric) form of the disease. CASE REPORT: This paper presents a 21 year old male patient with multicentric Castleman's disease, a transitional (mixed-cell) type. The disease was manifested by fever, generalized enlargement of peripheral lymph nodes, br

Long-lasting fever of unknown origin preceding the diagnosis of intravascular lymphomatosis: a further case stimulates some remarks.

Neurological and skin involvements usually dominate the clinical presentation of intravascular lymphomatosis (IL), while fever is the most frequent general sign. However, an onset only characterized by fever of unknown origin (FUO) has been rarely reported. We would like to describe a further case of IL, which presented a long-lasting FUO before the diagnosis. At admission, physical examination detected hepatosplenomegaly without lymph nodes enlargement or dermatological or neurological abnormalities. Significant laboratory data included severe anemia, leukopenia, thrombocytopenia, and increas

Lymphomatoid granulomatosis following autologous stem cell transplantation.

Lymphomatoid granulomatosis (LYG) is a rare angio-destructive lymphoproliferative disorder (LPD) of uncertain etiology, with prominent pulmonary involvement. Recent studies indicate that LYG is an Epstein-Barr virus (EBV)-associated B cell LPD with large numbers of background reactive T lymphocytes (T cell-rich B cell lymphoma). Although the disease frequently, but not exclusively, occurs in various immunodeficiency states, it has not been reported in association with the transient immunosuppression following autologous bone marrow/peripheral stem cell transplantation (ABM/PSCT). We describe a

[POEMS syndrome].

POEMS syndrome is an acronym defined by Bardwick (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal component and Skin changes). Other various clinical and biological features are reported: edema, cachexia, microangiopathic glomerulopathy, most rarely pulmonary hypertension, cutaneous necrosis. Thrombocytosis or polycythemia may be a prominent feature. POEMS syndrome is sometimes associated with lymphoproliferative disorder. Castelman-like disease is frequently observed as pathologic findings on lymph nodes. Distinction between POEMS syndrome and osteosclerotic myeloma is delicate. The

Castleman's disease associated with pemphigus vulgaris.

Castleman's disease is a rare, benign, lymphoproliferative disorder of unknown cause. The hyaline-vascular type is frequently associated with a localized mediastinal mass. The plasma-cell type is associated with constitutional symptoms, multicentric lymph node involvement, lymphoma development, and autoimmune disease-like laboratory abnormalities such as elevated erythrocyte sedimentation rate, anemia, and thrombocytopenia. We report a case of hyaline-vascular Castleman's disease associated with a cutaneous autoimmune disease, pemphigus vulgaris. We also reviewed the clinicopathologic features

Theileria parva: the nature of the immune response and its significance for immunoprophylaxis.

Theileria parva is a tick-borne haemoprotozoan parasite of cattle and buffalo which is responsible for considerable economic losses to cattle farming in Eastern, Central and Southern Africa. Infection with the parasite results in an acute lymphoproliferative disorder with high mortality, but animals which survive infection are solidly immune to homologous challenge. Such immunity can be reproduced by infecting cattle with the parasite and treating them with tetracyclines or theilericidal drugs, but the widespread use of this technique as a method of control is hindered by its dependence on liv

Path beyond the blind end-unravel the imaging spectrum of appendiceal pathologies.

The appendix is involved in a diverse spectrum of inflammatory, infectious, benign, and malignant conditions that extend far beyond acute appendicitis. Although acute appendicitis remains the most common appendiceal emergency, numerous entities-including reactive appendiceal inflammation, inflammatory bowel disease, appendiceal endometriosis, mucinous and non-mucinous neoplasms, lymphoma, post-transplant lymphoproliferative disorder, and rare mesenchymal tumors-may present with similar clinical symptoms and overlapping imaging findings. Accurate distinction among these conditions is essential

An archive that will have moved: on an exemplary artistic intervention in the archive Fritzpunkt, its relation to the Marianne Fritz archive in Vienna and its conceptual ramifications.

Prolymphocytic leukemia (PLL) is a rare lymphoproliferative disorder characterized by marked leukocytosis and splenomegaly. PLL accounts for approximately 2% of chronic lymphoid leukemias. The clinical course is progressive in the majority of cases due to the resistance of the disease to conventional chemotherapy. The disease is divided according to the cell of origin into the B- (B-PLL) and T-cell (T-PLL) types. T-PLL and B-PLL are morphologically identical, but lymphadenopathy and skin involvement are more common in T-PLL than in B-PLL. Approximately 80% of cases are of B-cell phenotype. T-P

Common connective tissue disorder and anti-cytokine autoantibodies are enriched in idiopathic multicentric castleman disease patients.

INTRODUCTION: Idiopathic Multicentric Castleman Disease (iMCD) is a polyclonal lymphoproliferative disorder involving cytokine storms that can lead to organ failure and death. The cause of iMCD is unknown, but some clinical evidence suggests an autoimmune etiology. For example, connective tissue disorders (CTDs) and iMCD share many clinical features, and autoantibodies have been anecdotally reported in individual iMCD patients. This study investigates whether common autoantibodies are shared across iMCD patients. METHODS: We assembled custom bead-based protein arrays consisting of 52 autoanti

Recommendations for the Management of Patients with Hairy-Cell Leukemia and Hairy-Cell Leukemia-like Disorders: A Work by French-Speaking Experts and French Innovative Leukemia Organization (FILO) Group.

INTRODUCTION: Hairy-cell leukemia (HCL) is a rare B-cell chronic lymphoproliferative disorder (B-CLPD), whose favorable prognosis has changed with the use of purine nucleoside analogs (PNAs), such as cladribine (CDA) or pentostatin (P). However, some patients eventually relapse and over time HCL becomes resistant to chemotherapy. Many discoveries have been made in the pathophysiology of HCL during the last decade, especially in genomics, with the identification of the BRAFV600E mutation and cellular biology, including the importance of signaling pathways as well as tumor microenvironment. All