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Sea-blue histiocytosis


Presentation

  • Abstract Splenic involvement by a light chain deposition disease (LCDD) associated with sea-blue histiocytosis occurred in a 55-year-old man presenting with LCDD of the kidney without myeloma.[ncbi.nlm.nih.gov]
Pathologist
  • The Color Atlas of Pediatric Pathology is a practical working resource for every pathologist who sees pediatric cases as well as the pathology trainee.[books.google.de]
High Fever
  • Case Report: A 40 year-old African-american male with a history of suicide attempt due to lye injestion, 10 years ago, with subsequent esophagogastrectomy, was admitted with high fevers and chills of one week duration.[nature.com]
Cutaneous Manifestation
  • The cutaneous manifestations appear as skin colored papules and grey pigmentation on the upper body. Occasionally, eyelid swelling can be observed.[perridermatology.com]
Subcutaneous Nodule
  • nodules / lipomas / tumefaction / swelling - Thrombocytopenia / thrombopenia Frequent - Lung / pulmonary infiltrates Occasional - Irregular / in bands / reticular skin hyperpigmentation - Retinopathy[csbg.cnb.csic.es]
Uremia
  • The association of congenital malformation and uremia might accentuate the symptoms.[ncbi.nlm.nih.gov]

Workup

Treatment

  • His symptoms did not resolve with the antibiotic treatment and further investigations revealed pulmonary involvement of sea-blue histiocytosis. After diagnostic bronchoalveolar lavage, his symptoms were improved, interestingly.[ncbi.nlm.nih.gov]
  • There are currently no formal treatment guidelines. Management may involve the coordinated care of several different specialists including cardiologists, gastroenterologists, and hematologists.[rarediseases.info.nih.gov]
  • Stay at the forefront of your field with updated treatment methods throughout, as well as an increased focus on patients with skin of color.[books.google.de]
  • Treatment - Sea-Blue histiocytosis Not supplied. Resources - Sea-Blue histiocytosis Not supplied.[checkorphan.org]
  • Center for Diagnosis and Treatment of Congenital Diseases of Metabolism, Burlo Garofalo Institute, Trieste, Italy[link.springer.com]

Prognosis

  • NPD type B is a rare inborn error of metabolism, with a benign course and prognosis, while types A and C are always associated with severe neurological involvement.[ncbi.nlm.nih.gov]
  • Prognosis - Sea-Blue histiocytosis Not supplied. Treatment - Sea-Blue histiocytosis Not supplied. Resources - Sea-Blue histiocytosis Not supplied.[checkorphan.org]
  • Prognosis and predictive factors The prognosis is usually favorable with a 10-year disease-related survival exceeding 90%.[epathologies.com]
  • NPD type A and C are seen in childhood and have poor prognosis. In SBH, hepatosplenomegaly is common. Other organ involvements are rare, as well neurological involvement is very rare ( 1 ). Our patient was 39-years-old.[tuberktoraks.org]

Etiology

  • Classification of these conditions often presents many nosological challenges, since underlying etiologies and pathogenetics are often not known.[howlingpixel.com]
  • The SBH syndrome is however not unique to NPD and histopathology lacks the specificity to make an etiologic diagnosis in most cases.[hanspopperhepatopathologysociety.org]
  • Images sea-blue histiocytes Synopsis bone marrow cytology (May-Giemsa staining) scattered foci containing aggregates of foamy macrophages blue-staining foamy macrophages coarse dark blue cytoplasmic granules light blue homogenous cytoplasmic staining Etiology[humpath.com]
  • […] cervical cancer in partners Protection from sexually transmitted infections, including human immunodeficiency virus infection (controversial) Reduced risk of urinary tract infections (controversial) Reduced risk of inflammatory genital skin diseases Etiologic[accessmedicine.mhmedical.com]
  • Primary cutaneous and primary nodal CD30 ALCL are distinct clinical entities that can have similar morphologic features and some overlap in immunophenotype, but differ in age of onset, genetic features, etiology and prognosis.[epathologies.com]

Epidemiology

  • […] variant Synonym(s): (no synonyms) Classification (Orphanet): - Rare genetic disease - Rare systemic or rheumatologic disease Classification (ICD10): - Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism - Epidemiological[csbg.cnb.csic.es]
  • Synonyms Regressing atypical histiocytosis , EORTC: Primary cutaneous large cell T cell lymphoma CD30 Epidemiology C-ALCL is the second most common form of cutaneous T-cell lymphoma with an incidence of 0.1-0.2 patients per 100’000.[epathologies.com]
  • Filipin stain is used on these cultured fibroblasts to visualize the accumulated free cholesterol and also to study the LDL-induced cholesterol ester formation. [8] The epidemiology of this disease in India is not known as yet.[neurologyindia.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology [ edit ] The high lipid content in the blood leads to excessive cytoplasm loading of lipids within histiocytes. The subsequent incomplete degradation of these lipids leads to the formation of cytoplasmic lipid pigments.[en.wikipedia.org]
  • Pathophysiology and presentation The high lipid content in the blood leads to excessive cytoplasm loading of lipids within histiocytes. The subsequent incomplete degradation of these lipids leads to the formation of cytoplasmic lipid pigments.[infogalactic.com]
  • Pathophysiology The high lipid content in the blood leads to excessive cytoplasm loading of lipids within histiocytes. The subsequent incomplete degradation of these lipids leads to the formation of cytoplasmic lipid pigments.[wikiwand.com]

Prevention

  • Prevention - Sea-Blue histiocytosis Not supplied. Diagnosis - Sea-Blue histiocytosis The signs and symptom information on this page attempts to provide a list of some possible signs and symptoms of Sea-Blue histiocytosis.[checkorphan.org]

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