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Internal Medicine (Tokyo, Japan) May 1996A 39-year-old male was admitted with fever, systemic lymph node swelling, liver dysfunction and mild splenomegaly. Liver biopsy specimen showed histiocytic aggregation... (Review)
Review
A 39-year-old male was admitted with fever, systemic lymph node swelling, liver dysfunction and mild splenomegaly. Liver biopsy specimen showed histiocytic aggregation in portal areas. These histiocytes were closely packed with granules, dyed sea-blue with May-Giemsa staining. Further microscopical examination of lymph nodes, gastro-intestinal tract and bone marrow also revealed the accumulation of sea-blue histiocytes. Activities of lipid metabolic enzymes were normal and hematopoietic diseases which are sometimes accompanied by secondary sea-blue histiocytosis were ruled out. We diagnosed this case as syndrome of the sea-blue histiocyte.
Topics: Adult; Humans; Leukocytes; Lipid Metabolism; Liver; Lymph Nodes; Male; Sea-Blue Histiocyte Syndrome
PubMed: 8797061
DOI: 10.2169/internalmedicine.35.419 -
Journal of Clinical Pathology Nov 1972The syndrome of sea-blue histiocyte, hepatic porphyria, and infectious mononucleosis occurring simultaneously in a young girl is described. The cytochemical staining...
The syndrome of sea-blue histiocyte, hepatic porphyria, and infectious mononucleosis occurring simultaneously in a young girl is described. The cytochemical staining character of the bone marrow histiocytes strongly suggested that the storage material was glycolipid. The possible relationship between the multiple disorders in the same patient is discussed.
Topics: Adolescent; Female; Glycolipids; Histiocytes; Histocytochemistry; Humans; Infectious Mononucleosis; Porphyrias; Splenomegaly; Staining and Labeling
PubMed: 4119308
DOI: 10.1136/jcp.25.11.945 -
The European Respiratory Journal Sep 1995The authors present the case of a 73 year old man with primary myelofibrosis, pulmonary fibrosis, and acquired sea-blue histiocytosis, who died of respiratory failure....
The authors present the case of a 73 year old man with primary myelofibrosis, pulmonary fibrosis, and acquired sea-blue histiocytosis, who died of respiratory failure. Pathology of the lungs revealed infiltration by sea-blue histiocytes, and fibrosis in the alveolar septa, and clumps of these cells filling the alveolar spaces. Megakaryocytes were also occasionally observed in the alveolar capillaries.
Topics: Aged; Biopsy; Fatal Outcome; Humans; Male; Primary Myelofibrosis; Pulmonary Fibrosis; Sea-Blue Histiocyte Syndrome
PubMed: 8575594
DOI: No ID Found -
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 -
Haematologica Jan 2021
Topics: Bone Marrow; Humans; Sea-Blue Histiocyte Syndrome
PubMed: 33386710
DOI: 10.3324/haematol.2020.273755 -
The Journal of Pediatric Pharmacology... 2015A syndrome of hepatosplenomegaly, thrombocytopenia, and anemia and the presence of sea-blue histiocytes in bone marrow has been associated with parenteral soybean oil...
A syndrome of hepatosplenomegaly, thrombocytopenia, and anemia and the presence of sea-blue histiocytes in bone marrow has been associated with parenteral soybean oil administration in patients receiving long-term total parenteral nutrition (TPN). A case is described here where this syndrome was observed in a pediatric patient who received long-term parenteral fish oil nutrition.
PubMed: 26170774
DOI: 10.5863/1551-6776-20.3.217 -
Journal of Clinical Pathology Nov 1993To determine whether myelodysplastic syndromes (MDS) are associated with sea blue histiocytosis in the bone marrow.
AIMS
To determine whether myelodysplastic syndromes (MDS) are associated with sea blue histiocytosis in the bone marrow.
METHODS
A retrospective review of bone marrow aspirates from 35 patients presenting consecutively with MDS and from 20 patients with each of the following: normal marrow appearance (routine staging for non-Hodgkin's lymphoma), polycythaemia rubra vera, immune thrombocytopenic purpura (ITP), chronic myeloid leukaemia (CML) in chronic phase.
RESULTS
Sea blue histiocytes were present in the marrow in 12 of 35 cases of MDS and occurred in large numbers in three of these cases. Sea blue histiocytes varied in the degree of cytoplasmic granularity and some cells were intermediate in appearance between classic sea blue histiocytes and pseudo-Gaucher cells. Sea blue histiocyte granules additionally stained positively with Sudan black and periodic acid schiff. Sea blue histiocytes occurred only in the presence of marrow hypercellularity. Their presence did not correlate with FAB subtype, degree of dyserythropoeisis, or megakaryocyte numbers. No sea blue histiocytes were found in the normal marrow or polycythaemia cases. In ITP sea blue histiocytes were seen in two of 20 cases and in chronic myeloid leukaemia in eight of 20 cases.
CONCLUSIONS
Sea blue histiocytes are a common cytological feature in the bone marrow of patients with MDS. As other disorders frequently associated with marrow sea blue histiocytes are relatively rare MDS is probably the most common cause of this phenomenon in a northern European population.
Topics: Aged; Aged, 80 and over; Bone Marrow; Female; Humans; Leukemia, Myelogenous, Chronic, BCR-ABL Positive; Male; Middle Aged; Myelodysplastic Syndromes; Purpura, Thrombocytopenic, Idiopathic; Retrospective Studies; Sea-Blue Histiocyte Syndrome
PubMed: 8254090
DOI: 10.1136/jcp.46.11.1030 -
Blood May 1971
Topics: Bone Marrow; Bone Marrow Cells; Cytoplasmic Granules; Female; Histiocytes; Humans; Lipids; Microscopy, Electron; Middle Aged; Polymers; Prednisone; Purpura, Thrombocytopenic; Spleen
PubMed: 5102920
DOI: No ID Found -
Proceedings of the Royal Society of... Jan 1973
Topics: Adult; Dietary Fats; Female; Histiocytes; Humans; Metabolic Diseases; Splenomegaly; Syndrome
PubMed: 4690047
DOI: No ID Found -
Respiratory Medicine Dec 2000Niemann-Pick disease (NPD) is a rare, inherited, autosomal recessive, lipid storage disease. The pathognomonic intracellular accumulation of sphingomyelin results in the... (Review)
Review
Niemann-Pick disease (NPD) is a rare, inherited, autosomal recessive, lipid storage disease. The pathognomonic intracellular accumulation of sphingomyelin results in the production and accumulation of 'foam cells'. Interstitial lung disease is a rare manifestation of NPD. We present the case of a 48-year-old white female with NPD involving the lungs, liver and spleen. The chest radiograph showed bilateral, predominantly basal reticulonodular infiltrates and serial pulmonary function tests over a period of years showed preserved expiratory airflow and a severely decreased diffusion capacity for carbon monoxide (DLCO). In view of her visceral involvement, lack of neurological symptoms and survival into adulthood, we believe our patient represents a case of type B NPD. In this type of NPD, aside from prominent hepatosplenomegaly and sexual immaturity, significant pulmonary infiltration with 'Pick cells' has been reported. To date, no therapeutic modality has been shown to alter the natural history of this disease, which results in progressive debilitation and death. This case is unique in that it provides the longest physiological follow-up in the literature, and provides data on the natural history of pulmonary involvement in NPD.
Topics: Carbon Monoxide; Diffusion; Female; Humans; Lung Diseases, Interstitial; Middle Aged; Niemann-Pick Diseases; Radiography; Respiratory Function Tests; Sea-Blue Histiocyte Syndrome
PubMed: 11192962
DOI: 10.1053/rmed.2000.0942