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]
Diebold 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. [link.springer.com]
Accumulation of sea blue histiocytes in the cords was also present. Stiffness of the walls of the red pulp sinuses resulting from light chain deposits may have induced… CONTINUE READING [semanticscholar.org]
Entire Body System
- Refractory Anemia
anemia with excess blasts Refractory anemia with excess blasts in transformation Electron microscopy description Electron microscopy images Images hosted on other servers : Hyperlipidemia: cytoplasmic electron dense granules "Finger print" arrangement [pathologyoutlines.com]
- 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]
Respiratoric
- Productive Cough
He was admitted to our clinic due to productive cough, hemoptysis, fever and weight loss. His symptoms did not resolve with the antibiotic treatment and further investigations revealed pulmonary involvement of sea-blue histiocytosis. [ncbi.nlm.nih.gov]
Urogenital
- Uremia
The association of congenital malformation and uremia might accentuate the symptoms. [ncbi.nlm.nih.gov]
Workup
Clinical History A 30 year old man was referred for liver transplantation workup for presumed liver cirrhosis from NASH. [hanspopperhepatopathologysociety.org]
Biopsy
- Bone Marrow with Sea-Blue Histiocytes
Aspirated bone marrows contained sea-blue histiocytes, and nitroblue tetrazolium test was abnormal. X-ray examination revealed a tumor mass in the right lung, uremia occurred terminally, and he died at the age of 10 years and 3 months. [ncbi.nlm.nih.gov]
Classically, the bone marrow shows “sea-blue histiocytes,” which are macrophages with abundant blue-green to blue granular cytoplasmic inclusions (see Fig. 7.9C ). [obgynkey.com]
Treatment
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]
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]
Today’s breakthrough treatment can quickly become tomorrow’s unexpectedly risky treatment. Information comes out quickly, sometimes even before it has been thoroughly thought out. [savemoney.es]
Although non-specific antibiotic treatment of amoxicillin-clavulanic acid and clarithromycin was administered, no clinical or radiological improvement was achieved. [tuberktoraks.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 and predictive factors The prognosis is usually favorable with a 10-year disease-related survival exceeding 90%. [epathologies.com]
Prognosis - Sea-Blue histiocytosis Not supplied. Treatment - Sea-Blue histiocytosis Not supplied. Resources - Sea-Blue histiocytosis Not supplied. [checkorphan.org]
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
The SBH syndrome is however not unique to NPD and histopathology lacks the specificity to make an etiologic diagnosis in most cases. [hanspopperhepatopathologysociety.org]
Classification of these conditions often presents many nosological challenges, since underlying etiologies and pathogenetics are often not known. [howlingpixel.com]
/status/748512208993271808 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]
Pathophysiology
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 [ 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 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]