Search results for "Histiocytosis"

Inflammatory pituitary lesions

2021

Abstract Inflammatory pituitary lesions are rare but important differential diagnoses of nonfunctioning pituitary masses. Primary hypophysitis as an autoimmune disorder has received increasing attention over the past decades. In the last decade, immune checkpoint inhibitor–induced hypophysitis has emerged as a new type of secondary hypophysitis. Various pituitary tumors may also cause secondary hypophysitis. Systemic, noninfectious granulomatous diseases that can involve the pituitary region include sarcoidosis, granulomatosis with polyangiitis, Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease. Furthermore, infectious diseases can affect the pituitary gland.…

Sepsis due to Streptococcus pneumoniae associated with secondary hemophagocytic lymphohistiocytosis in a splenectomized patient for spherocytosis

2017

Abstract Rationale: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome that is characterized by an inappropriate hyperinflammatory immune response – primary, as a consequence of a genetic defect of NK cells and cytotoxic T lymphocytes or – secondary, in the progression of infections, rheumatic or autoimmune diseases, malignancies or metabolic diseases. Patient concerns: We present the case of a secondary HLH due to Streptococcus pneumoniae infection in a splenectomised patient for spherocytosis, a 37-year-old patient who was splenectomised in childhood for spherocytosis, without immuneprophylaxis induced by antipneumococcal vaccine. Outcomes: He developed a severe pneumococcal sepsis as…

Secondary hemophagocytic lymphohistiocytosis: forget me not!

2012

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Canine pancytopoenia and hemophagocytic lymphohistiocytosis.

2018

Leishmaniasis and rickettsial diseases are endemic and common in Mediterranean countries and so, as in humans, these diseases could be an important cause of HLH in dogs and may respond to similar medications. In conclusion, we think that the diagnosis of HLH should be considered in dogs with pancytopenia and, in selected cases, immunosuppressive treatment might be warranted.

Bifocal manifestation of eosinophilic granuloma in a paediatric patient-A case report

2009

Background: Eosinophilic granuloma (EG) is a clinical variant of the Langerhans cell histiocytosis (LCH) characterized by unifocal or multifocal bone lesions which predominantly affects children, adolescents, and young adults. Case Report: A case is reported of a 13-year-old Caucasian boy who presented unifocal EG in the mandible as the first clinic manifestation. Radiographic examination and skeletal scintigraphy revealed a further localization with an osteolytic lesion in the right femur. The therapeutic protocol used for the mandibular lesion included causal periodontal therapy, extraction of the compromised teeth, alveolar curettage, and intralesional injections of corticosteroids, in c…

Secondary hemophagocytic lymphohistiocytosis in zoonoses. A systematic review

2012

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome that is often fatal despite treatment. It is caused by a dysregulation in natural killer T-cell function, resulting in activation and proliferation of histiocytes with uncontrolled hemophagocytosis and cytokines overproduction. The syndrome is characterized by fever, hepatosplenomegaly, cytopenias, liver dysfunction, and hyperferritinemia. HLH can be either primary, with a genetic aetiology, or secondary, associated with malignancies, autoimmune diseases, or infections. AIM: To focus on secondary HLH complicating zoonotic diseases. MATERIALS AND METHODS: PubMed search of human cases of HLH occurring during zoonotic dise…

Alternative UNC13D Promoter Encodes a Functional Munc13-4 Isoform Predominantly Expressed in Lymphocytes and Platelets

2020

Autosomal recessive mutations in genes required for cytotoxicity are causative of a life-threatening, early-onset hyperinflammatory syndrome termed familial hemophagocytic lymphohistiocytosis (FHL). Mutations in UNC13D cause FHL type 3. UNC13D encodes Munc13-4, a member of the Unc13 protein family which control SNARE complex formation and vesicle fusion. We have previously identified FHL3-associated mutations in the first intron of UNC13D which control transcription from an alternative transcriptional start site. Using isoform specific antibodies, we demonstrate that this alternative Munc13-4 isoform with a unique N-terminus is preferentially expressed in human lymphocytes and platelets, as…

Sinus histiocytosis with massive lymphadenopathy : is the lymph node enlargement always massive?

2007

Sinus histiocytosis with massive lymphadenopathy (SHML) is a benign proliferating histiocytic disorder, predominantly of lymph nodes with extra-nodal involvement in some cases. It is a self-limiting disease and has a good prognosis; however some patients need steroid therapy. Therein lays a need to differentiate it from other lympho proliferative disorders of poorer prognosis. Clinically, it is suspected only when there is a significant lymph node enlargement in younger age group as the term signifies. Recently, we came across a case where a middle-aged female presented with a subtle swelling in submental region, clinically suspected of reactive or tubercular etiology. However on fine needl…

Tropical diseases in the ICU: Please do not forget hemophagocytic lymphohistiocytosis

2018

A rare case of oral multisystem Langerhans cell histiocytosis

2017

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by high proliferation of Langerhans dendritic cells. LCH is a solitary or multifocal disease that primarily involves bone tissue and often affects children and young men. A 29 years-old Caucasian man was referred to the Oral Surgery Unit of George Eastman Hospital - Umberto I teaching hospital, with third degree mobility of teeth belonging to second, third and fourth quadrant. Panoramic radiograph showed multiple radiolucent areas with well demarcated borders on the right and left site of the mandible and on the left site of the maxilla. Extractions of compromised teeth and biopsy of the osteolytic tissue were performed. T…