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Nigerian Journal of Clinical Practice Nov 2019Budd-Chiari syndrome (BCS) is a rare disease characterized by obstruction of hepatic venous outflow tract with diversified etiologies. Sea-blue histiocytosis (SBH) is a...
Budd-Chiari syndrome (BCS) is a rare disease characterized by obstruction of hepatic venous outflow tract with diversified etiologies. Sea-blue histiocytosis (SBH) is a kind of storage diseases defined by the deposition of abundant sea-blue histiocytes in various organs and can lead to hepatosplenomegaly, cirrhosis, or even liver failure. The association between BCS and SBH has never been reported before. Here, we report a patient with BCS presenting with hepatosplenomegaly, portal hypertension, and pancytopenia who was later confirmed to also have SBH.
Topics: Adult; Budd-Chiari Syndrome; Hepatomegaly; Humans; Hypertension, Portal; Male; Pancytopenia; Rare Diseases; Sea-Blue Histiocyte Syndrome; Splenomegaly; Vena Cava, Inferior
PubMed: 31719286
DOI: 10.4103/njcp.njcp_43_19 -
The Journal of the Association of... Apr 1982
Topics: Adolescent; Female; Histiocytes; Humans; Lymphatic Diseases; Syndrome
PubMed: 7182396
DOI: No ID Found -
Yonsei Medical Journal 1983
Topics: Child, Preschool; Humans; Hyperlipoproteinemia Type II; Male; Sea-Blue Histiocyte Syndrome; Spleen
PubMed: 6679679
DOI: 10.3349/ymj.1983.24.2.132 -
Journal of Clinical and Experimental... Apr 2007Sea-blue histiocytosis is a rare disorder seen in patients with lipid metabolic or ceroid storage diseases. Sea-blue histiocytes are ceroid-laden macrophages detectable...
Sea-blue histiocytosis is a rare disorder seen in patients with lipid metabolic or ceroid storage diseases. Sea-blue histiocytes are ceroid-laden macrophages detectable by May-Giemsa staining. We report a case of a 28-year-old woman diagnosed with Niemann-Pick disease at 2 or 3 years of age. To confirm this diagnosis, we examined her bone marrow, which revealed scattered foci containing aggregates of foamy macrophages. May-Giemsa staining identified blue-staining foamy macrophages, referred to as sea-blue histiocytes. In summary, we report the detection of sea-blue histiocytosis in an adult with Niemann-Pick disease.
Topics: Adult; Bone Marrow Cells; Bone Marrow Examination; Female; Humans; Immunohistochemistry; Leukocytes; Macrophages; Niemann-Pick Diseases; Sea-Blue Histiocyte Syndrome
PubMed: 17510534
DOI: 10.3960/jslrt.47.19 -
Sbornik Vedeckych Praci Lekarske... 1975
Topics: Adult; Biopsy; Hepatomegaly; Histiocytes; Humans; Liver; Male; Syndrome
PubMed: 1071837
DOI: No ID Found -
American Journal of Hematology May 2024Bone marrow smear showing histiocytes (black arrow) containing sea blue granules stained with May-Grünwald Giemsa.
Bone marrow smear showing histiocytes (black arrow) containing sea blue granules stained with May-Grünwald Giemsa.
Topics: Humans; Sea-Blue Histiocyte Syndrome; Pancytopenia; Parenteral Nutrition; Histiocytes
PubMed: 38115676
DOI: 10.1002/ajh.27185 -
Annals of Hematology Oct 2001Sea-blue histiocytosis is a morphological finding that can be associated both with acquired conditions of increased cellular turnover and inborn errors of lipid...
Sea-blue histiocytosis is a morphological finding that can be associated both with acquired conditions of increased cellular turnover and inborn errors of lipid metabolism. We report a rare case of sea-blue histiocytosis associated with a mild phenotype of Niemann-Pick disease (NPD) type B in a 44-year-old man who presented with splenomegaly and mild thrombocytopenia. Diagnosis was guided by the morphological finding in bone marrow smears of foamy and sea-blue histiocytes and confirmed by the measurement of acid lysosomal sphingomyelinase activity below normal values. 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. In our patient diagnosis was confirmed by the specific enzyme assay of leukocytes (deficiency in sphingomyelinase activity). This is a simple and noninvasive method that is useful whenever clinical and morphological finding are relevant, and a primary hematological disorder has been ruled out.
Topics: Adult; Humans; Leukocytes; Lysosomes; Male; Niemann-Pick Diseases; Sea-Blue Histiocyte Syndrome; Sphingomyelin Phosphodiesterase; Splenomegaly; Thrombocytopenia
PubMed: 11732877
DOI: 10.1007/s002770100354 -
Revista Clinica Espanola Oct 1985
Topics: Adult; Female; Fever of Unknown Origin; Hepatomegaly; Humans; Sea-Blue Histiocyte Syndrome
PubMed: 4081199
DOI: No ID Found -
Archives of Pathology Jun 1972
Topics: Adult; Cell Nucleus; Cytoplasmic Granules; Female; Histiocytes; Humans; Inclusion Bodies; Lipidoses; Male; Microscopy, Electron; Spleen; Splenic Diseases; Staining and Labeling
PubMed: 4112533
DOI: No ID Found -
Annals of Clinical Laboratory Science 1972
Topics: Adult; Histiocytes; Histocytochemistry; Humans; Male; Niemann-Pick Diseases; Pigments, Biological; Spleen; Staining and Labeling; Syndrome
PubMed: 4117249
DOI: No ID Found