536
BRIEF
REPORTS
SYSTEMIC
MASTOCYTOSIS
AFFECTING
J. V. COOK, J. CHANDY
THE
SKELETAL
SYSTEM
Case report. A 56-year-old dryness of her skin, prunitus was noted to have marked an Routine renal investigations failure and
woman was and attacks cyanosis that
being treated of flushing. and she was urogram
for She feet.
of her hands
revealed intravenous
in mild showed
multiple sclerotic bone lesions in the lumbar spine and right femoral neck (Figs 1 and 2). A skin biopsy stained with Giemsa, showed mast cells in excess of the normal number and histology of the femoral neck was also interpreted as being consistent with mastocytosis. Treatment with was histamine commenced. her prunitus blockers (cimetidine and cyproheptadine) improved remains. Discussion. characterised Systemic mastocytosis is a rare by infiltration of various organs condition by mast
Fig. 1 Fig. 2
A two-month course has so far but her unusual acrocyanosis
cells. Benign and malignant forms are described, the benign existing in cutaneous and systemic varieties. The cutaneous form is mostly seen in childhood. The mast cell naevus is usually present at birth, while the more generalised urticania pigmentosa occurs at about three to nine 10% tions, months. Both forms are of the latter may progress Adults but usually 50% present progress to usually self-limiting, to systemic mastocytosis. with the cutaneous systemic manifestaform of the but
The histological diagnosis depends on the special metachromatic staining of the distinctive mast cell granules with Giemsa or toluidine blue. The biopsy specimens need careful handling to prevent degranulation. Because of the patchy difficulties with staining, often difficult to obtain as well and uptake and metabolites Technetium generalised and gallium nature positive 24-hour bone scans Jackson taken up of the disease and marrow biopsies urine scanning demonstrate and by the Reid mast collections are the are for useful. diffuse 1983) cell.
as isotope gallium (Ensslen,
disease which affects gastrointestinal tract. malignant transformation et al.
skin, bone, There is to mast
liver, spleen and the a 5% incidence of cell leukaemia (Rafli
is particularly
1983), which responds poorly to treatment. Mast cell proliferation particularly affects the reticulo-endothelial system. The cells secrete a variety of pharmacologically active agents, including histamine, hepanin, prostagland ins, serotonin and mucopolysacThese substances of presentations abnormalities play an important of the disease. have been described role in the in 10%
In conclusion, systemic mastocytosis should be considered in patients with painless sclerotic bone lesions or progressive osteoporosis. The importance of a skeletal survey and isotope bone scanning in patients with persistent urticarial skin conditions is stressed as is the careful handling of biopsy specimens.
chanides. variety
Skeletal
of patients with systemic mastocytosis. Osteoporosis has been thought to be secondary to chronic heparin release and secretion of prostaglandin D2 by the mast cell. The latter may also be significant in the cause of acrocyanosis in the case presented (Kootte, Haak and Roberts 1983).
We would like to thank Drs Dukes and Ashworth for their help with the case material. Also Mrs P. Vecchi for help with the preparation of the manuscript. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.
REFERENCES J. V. Cook, St. Georges MRCP, Hospital, FRCR, Senior Registrar in Radiology Blackshaw Road, London SWI7, England. FRCR, Clifford England. to Dr Consultant Radiologist Bridge Road, Walsgrave, Coventry CV2
J. Chandy, DMRD, Walsgrave Hospital, 2DX, Warwickshire, Correspondence
Ensslen RD, Jackson Fl, Reid AM. Bone gallium correlation with count rates, radiography, Med 1983:24:586-8. Kootte
scans in mastocytosis: and microscopy. J Nuc/ : an expression D2 produc-
J. V. Cook. of Bone and Joint Surgery
AMM, Haak A, Roberts LI. The flush syndrome of systemic mastocytosis with increased prostaglandin tion. Neth J Med 1983:26:18-9.
) 1989 British Editorial Society 0301 620X/89/3R07 $2.00 J Bone Joint Surg (Br] 1989:71-B:536.
Rafli M, Firooznia
systemic literature.
H, Golimbu
C, Balthazar
E. Pathological
and
fracture
review
in
mastocytosis C/in Orthop
: radiographic spectrum 1983:180:260-7.
of the
TIlE
JOURNAL
OF
BONE
AND
JOINT
SURGERY