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The American Journal of Medicine Mar 1978A middle-aged man with lymphocytic lymphoma had numerous sea-blue histiocytes in his bone marrow, splenomegaly and thrombocytopenia. Thus, his illness mimicked that of...
A middle-aged man with lymphocytic lymphoma had numerous sea-blue histiocytes in his bone marrow, splenomegaly and thrombocytopenia. Thus, his illness mimicked that of patients with the primary syndrome of the sea-blue histiocyte. However, the paucity of sea-blue histiocytes in his spleen, the absence of neurologic disease, his age and the ultrastructure of his abnormal histiocytes were all evidence for the presence of the acquired syndrome. The pathogenesis of sea-blue histiocytosis and the relationship between acquired cases and the primary syndrome are discussed.
Topics: Biopsy; Bone Marrow; Histiocytes; Humans; Lymphocytes; Lymphoma, Non-Hodgkin; Lysosomes; Male; Middle Aged; Splenomegaly; Staining and Labeling; Syndrome
PubMed: 76448
DOI: 10.1016/0002-9343(78)90245-0 -
Tuberkuloz Ve Toraks 2012Sea-blue histiocytosis is one of the six types of Niemann-Pick disease. It is characterized by childhood onset of hepatosplenomegaly, lack of neurological involvement...
Sea-blue histiocytosis is one of the six types of Niemann-Pick disease. It is characterized by childhood onset of hepatosplenomegaly, lack of neurological involvement and diminished sphingomyelinase activity. Pulmonary system is rarely involved sea-blue histiocytosis. In this paper, we present a 39-years-old male who had previously diagnosed as sea-blue histiocytosis at the age of 15. 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. After diagnostic bronchoalveolar lavage, his symptoms were improved, interestingly. This rare entity was discussed with literature survey.
Topics: Adult; Bronchoalveolar Lavage; Humans; Lung Diseases; Male; Sea-Blue Histiocyte Syndrome; Treatment Outcome
PubMed: 22779941
DOI: 10.5578/tt.2215 -
Acta Haematologica 1977We report two cases of sea-blue histiocyte syndrome in Thai siblings. The abnormal histiocytes were found in the bone marrow and liver of both patients, but none was...
We report two cases of sea-blue histiocyte syndrome in Thai siblings. The abnormal histiocytes were found in the bone marrow and liver of both patients, but none was found in the other members of the family. Cirrhosis and absence of axillary hair were present in both patients. One patient also had elephantiasis of the legs and Klinefelter's syndrome. Our studies support an autosomal recessive inheritance.
Topics: Bone Marrow; Hepatomegaly; Histiocytes; Liver; Liver Cirrhosis; Splenomegaly; Syndrome; Thailand
PubMed: 410219
DOI: 10.1159/000207807 -
Nutricion Hospitalaria 2009A case of a 55 years-old male with long-term Crohn's disease without response to medical treatment and many intestinal fistula is presented. After the last bowel...
A case of a 55 years-old male with long-term Crohn's disease without response to medical treatment and many intestinal fistula is presented. After the last bowel resection, home parenteral nutrition was started. He presented chronic hepatopathy and pancytopaenia. After 9 months of home parenteral nutrition hepatic function and pancytopaenia began to deteriorate. Bone marrow examination revealed an infiltrate of sea-blue histiocytes. He made unsatisfactory progress and died due to a multiorganic failure.
Topics: Humans; Male; Middle Aged; Parenteral Nutrition, Home; Sea-Blue Histiocyte Syndrome
PubMed: 19721912
DOI: No ID Found -
Medicina Clinica Sep 1979The literature published before October, 1977 on the so-called sea-blue histiocyte syndrome is reviewed. This is a new lipid thesaurismosis, and from a morphological...
The literature published before October, 1977 on the so-called sea-blue histiocyte syndrome is reviewed. This is a new lipid thesaurismosis, and from a morphological point of view it is characterized by the appearance of large histiocytes in the organs of the reticuloendothelial system with numerous intracytoplasmic granules which take on a typical sea-blue or greenish color with Wright's or Giemsa stain. The exact nature of the accumulated substance has not yet been specifically determined, though it appears to be gluco- and/or phosphosphingolipid, essentially sphingomyelin. The specific biochemical alteration responsible for this chronic deposit has not been established, though a partial sphingomyelinase deficiency has been detected. Sea-blue histiocytes have been observed in two different situations, either as an acquired phenomenon or as a primary condition. Of the latter there have been sporadic cases and cases with a definite familial incidence. The clinical manifestations include enlargement of the liver and spleen, neurological symptoms, cirrhosis of the liver, hemorrhagic diathesis and purpura, chronic pneumopathies, eye or cutaneous disturbances, or no symptoms at all. The disease has a benign clinical course, and the prognosis is less favourable when clinical manifestations appear early in life. These cases have a greater tendency to develop neurological alterations. The final definition of the syndrome must await the clear identification of the accumulated material and the altered enzyme or metabolic pathway.
Topics: Diagnosis, Differential; Histiocytes; Humans; Niemann-Pick Diseases
PubMed: 491788
DOI: No ID Found -
Neurology Nov 1975This is a report of a new familial neurologic disorder characterized by ceroid-lipofuscin storage, sea-blue histiocytes, and associated neurologic findings. Neurologic...
This is a report of a new familial neurologic disorder characterized by ceroid-lipofuscin storage, sea-blue histiocytes, and associated neurologic findings. Neurologic manifestations in the family members examined were varied, but posterior column involvement was the most common finding. The presence of sea-blue histiocytes was genetically determined by an autosomal dominant trait. Vitamin E blood concentrations were decreased in the absence of absorption difficulties in two sisters who were most seriously affected. The relationship of ceroid-lipofuscin, lipid peroxidation, and vitamin E is discussed.
Topics: Adolescent; Adult; Ceroid; Chromosome Aberrations; Chromosome Disorders; Female; Genes, Dominant; Histiocytes; Histocytochemistry; Humans; Lipidoses; Lipofuscin; Male; Pedigree; Syndrome; Vitamin E
PubMed: 1237827
DOI: 10.1212/wnl.25.11.1084 -
Haematologica Jan 2021
Topics: Bone Marrow; Humans; Sea-Blue Histiocyte Syndrome
PubMed: 33386710
DOI: 10.3324/haematol.2020.273755 -
Lancet (London, England) Sep 1970
Topics: Histiocytes; Histocytochemistry; Humans
PubMed: 4195238
DOI: 10.1016/s0140-6736(70)91383-8 -
Lancet (London, England) Dec 1970
Topics: Adult; Bone Marrow Diseases; Eye Diseases; Histiocytes; Humans; Macula Lutea; Male
PubMed: 4098670
DOI: 10.1016/s0140-6736(70)92201-4 -
Acta Pathologica Japonica Jan 1979A case of the syndrome of sea-blue histiocyte is presented in a 53-year-old Japanese woman, which is the first recorded case in Japan. The patient had...
A case of the syndrome of sea-blue histiocyte is presented in a 53-year-old Japanese woman, which is the first recorded case in Japan. The patient had hepatosplenomegaly, bleeding manifestations, mild thrombocytopenia, fatty metamorphosis and cirrhosis of the liver, as well as abnormal serum lipid profiles. Her parents were consanguineous and her maternal grandmother with hepatomegaly died of hepatic failure. Histologically, peculiar histiocytes containing numerous, intracytoplasmic sea-blue stained granules on May-Giemsa stain were demonstrated in biopsy materials of the bone marrow, lymph node and liver. The sea-blue granules in these histiocytes proved to have histochemical staining characteristics of lipogenic ceroid-like pigment. Ultrastructurally, these granules showed membrane-bound, pleomorphic inclusions of heterogeneous nature, including electron-dense amorphous or variegatedly osmiophilic, frequently laminated materials. Enzyme cytochemically, localization of acid phosphatase activity was demonstrated in and around the intracytoplasmic inclusions. With regard to the pathogenesis of the sea-blue histiocytes in this case, it may be suggested that the existence of the abnormality in lipid metabolism plays an important role in intralysosomal ceroidogenesis in these histiocytes.
Topics: Acid Phosphatase; Bone Marrow; Female; Histiocytes; Histocytochemistry; Humans; Inclusion Bodies; Kupffer Cells; Liver; Lymphatic Diseases; Middle Aged; Syndrome; Triglycerides
PubMed: 219660
DOI: 10.1111/j.1440-1827.1979.tb01294.x