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sea-blue histiocytes

Wednesday 18 January 2006

Definition: Marcophages that accumulate ceroids and/or lipofuscin in their cytoplasm. They are associated with a high rate of intramedullary cell death or storage diseases. Sea-blue histiocytosis can be observed in patients with genetic lipid metabolic diseases or ceroid storage diseases.

This morphological finding can be associated both with acquired conditions of increased cellular turnover and inborn errors of lipid metabolism.

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

- thrombocytopenia

  • idiopathic thrombocytopenic purpura (ITP) (#6684881#, #6779489#)

- Niemann-Pick diseases (#17510534#, #1401826#)

  • Niemann-Pick disease type B (#11732877#, #16315787#, #16163596#, #14623788#, #11524264#)
  • Niemann-Pick disease type C (#11778157#, #1817035#)

- inherited lysosomal diseases

  • ceroid lipofuscinosis

- cellular proliferations

  • chronic myeloid leukemia (CML)
  • chronic myelomonocytic leukemia (#289428#) lymphoma (#76448#)
  • Hodgkin lymphoma
  • Langerhans cell histiocytosis
  • light chain deposition disease (#10389632#)
  • chronic myeloid leukaemia (CML) (#3192753#)

- thalassemias
- hepatic diseases
- total parenteral nutrition (#9613982#, #9352150#, #8904878#)
- infections

  • lepromatous leprosy (#16961654#)

- hypertriglyceridemia by mutations in the receptor-binding region of the apoE molecule (#16094309#, #11095479#)

- sea-blue histiocyte syndrome (SBHS) (#11221166#, #3803674#, #6511031#, #6299321#)
- adult cholesteryl ester storage disease (CESD) (#10551400#)
- GM1 gangliosidosis type 2 (#1588015#)
- partial sphingomyelinase deficiency (#2542678#)
- sphingomyelin lipidosis (#3085631#)
- Hallervorden-Spatz disease (#6681873#)
- cholesterol ester storage disease (#6478639#)
- active systemic lupus erythematosus (#8867368#)
- primary myelofibrosis (#8575594#)
- myelodysplastic syndromes (#8254090#)
- chronic granulomatous diseases (CGDs)

  • variant chronic granulomatous disease with residual monocyte NADPH-oxidase activity (#2064968#, #6524383#)

- sea-blue histiocytosis with progressive anterior horn cell and axonal degeneration (#6089645#)
- progressive neurologic disorder with supranuclear vertical gaze paresis (#6727430#)

According to type of staining

- coarse dark blue cytoplasmic granules

  • Hodgkin lymphoma
  • hepatic diseases
  • parenteral nutrition

- light blue homogenous cytoplasmic staining

  • chronic myeloid leukemia (CML)
  • Langerhans cell histiocytosis
  • thalassemias

Differential diagnosis

- Gaucher cells

See also

- medullary macrophages (medullary histiocytosis)

References

- Lee RE. Histiocytic diseases of bone marrow. Hematol Oncol Clin North Am. 1988 Dec;2(4):657-67. PMID: #3220804#

- Zwi LJ, Lampert IA. Lipofuscin accumulation in the human spleen with an unusual distribution. A case report. Virchows Arch A Pathol Anat Histopathol. 1986;409(1):119-24. PMID: 3085336#