Effects of Primary Mast Cell Disease on Hemostasis and Erythropoiesis

Mast cell disease is an epigenetically and genetically determined disease entity with very diverse clinical manifestations in potentially every system and tissue due to inap pro priate release of variable subsets of mast cell mediators together with accumulation of either morphologically normal or altered mast cells. Easy bruising, excessive bleeding, and aberrancies of erythropoiesis can frequently be observed in patients with mast cell disease. A thorough history, including a family history, will guide the appropriate work-up, and laboratory evaluations may provide clues to diagnosis. In recent years, our understanding of the involvement of coagulation and anticoagulant pathways, the fibrinolytic system, and erythropoiesis in the pathophysiology of mast cell disease has increased considerably. This review summarizes current knowledge of the impact of the disturbed hemostatic and erythropoietic balance in patients with mast cell disease and describes options of treatment.

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Expression of lymphoid-associated antigens in mast cells: report of a case of systemic mast cell disease

British Journal of Haematology ◽

10.1111/j.1365-2141.1995.tb05417.x ◽

1995 ◽

Vol 91 (4) ◽

pp. 941-943 ◽

Cited By ~ 30

Author(s):

Luis Escribano ◽

Alberto Orfao ◽

Jesús Villarrubia ◽

Carlos Cerveró ◽

José L. Velasco ◽

...

Keyword(s):

Mast Cell ◽

Mast Cells ◽

Cell Disease ◽

Mast Cell Disease

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Radiotherapy of Refractory Bone Pain Due to Systemic Mast Cell Disease

American Journal of Clinical Oncology ◽

10.1097/00000421-199408000-00009 ◽

1994 ◽

Vol 17 (4) ◽

pp. 328-330 ◽

Cited By ~ 19

Author(s):

Peter A. S. Johnstone ◽

JoAnn M. Mican ◽

Dean D. Metcalfe ◽

Thomas F. DeLaney

Keyword(s):

Mast Cell ◽

Bone Pain ◽

Cell Disease ◽

Mast Cell Disease

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GENERALIZED MAST CELL DISEASE AND URTICARIA PIGMENTOSA

PEDIATRICS ◽

10.1542/peds.19.6.1023 ◽

1957 ◽

Vol 19 (6) ◽

pp. 1023-1032

Author(s):

Thomas L. Rider ◽

Arthur A. Stein ◽

John W. Abbuhl

Keyword(s):

Mast Cell ◽

Fibrous Tissue ◽

Cell Activity ◽

Gastrointestinal Symptoms ◽

Urticaria Pigmentosa ◽

Cell Infiltration ◽

Cell Disease ◽

Tissue Proliferation ◽

Bone Changes ◽

Mast Cell Disease

The case which is presented and review of the literature indicate that urticaria pigmentosa may be accompanied by mast cell infiltration of many tissues and viscera. No definite conclusions may be drawn regarding etiology, incidence, or prognosis of this disorder. The evidence indicates that both local and generalized symptoms occur which are principally related to the pathophysiologic changes resulting from mast cell activity, i.e., fibrous tissue proliferation, hyperemia and edema. In the case reported herein there was no histologic evidence of fibrous tissue increase but it is postulated that the hepatosplenomegaly and the bone changes in roentgenograms may be in part due to such changes. The dermatographism, skin flushing, salivary gland swelling and gastrointestinal symptoms are probably due to the physiologic action of mast cell products, i.e., histamine and serotonin. The diagnosis of generalized mast cell disease can be made in a patient who presents a chronic maculopapular skin rash, dermatographism, hepatosplenomegaly and mast cell infiltration of the bone marrow. Demonstration of mast cell infiltration in the skin and other tissues is confirmatory but not necessary.

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