There is currently no cure for mastocytosis. The treatment of mastocytosis
is generally conservative and symptomatic and is designed to prevent or ame
liorate the deleterious effects of mast cell mediators rather than to elimi
nate the mast cells which produce and release them.(9) Thus, management of
mastocytosis begins with educating the patient to avoid specific factors th
at can cause the release of mast cell mediators and the symptomatic respons
es to them. Direct therapeutic intervention is tailored to the symptom comp
lex of individual patients and consists mainly of drugs that block histamin
e receptors. Blockage of H-1 receptors has some efficacy in controlling cut
aneous symptoms such as flushing and itching, and H-2 antagonists are used
to prevent and treat histamine-induced peptic ulcer disease, cramping, and
diarrhea. Disodium cromoglycate stabilizes mast cell membranes, decreases m
ediator release, and seems to be helpful in relieving cramping and diarrhea
in some patients, but its use is controversial. Besides these extremely ag
gravating symptoms, which can make life miserable for mastocytosis patients
, serious problems such as life-threatening anaphylaxis may be associated w
ith the release of mast cell mediators. Adrenaline and full supportive care
may be necessary for anaphylaxis or vascular collapse induced by massive m
ast cell degranulation. Furthermore, although the contribution of mast cell
s and their mediators to the pathogenesis of the myelofibrotic and myelodys
plastic bone marrow disease, severe anemia, and hematologic malignancy asso
ciated with mastocytosis has not been determined, it is possible that the m
ast cells directly contribute to these life-threatening conditions. Obvious
ly, it would be preferable to treat mastocytosis by decreasing or eliminati
ng the number of neoplastic mast cells. Several forms of therapy have been
used to decrease mast cell numbers temporarily, but these therapies are ass
ociated with significant adverse side effects. Psoralen-ultraviolet A thera
py (PUVA) can decrease the number of cutaneous mast cells and suppress prur
itus. Psoralen-ultraviolet A therapy may also have cosmetic benefits for pa
tients with cutaneous mastocytosis, but its long-term use is associated wit
h an increased risk of skin cancer, whereas its therapeutic benefits are on
ly temporary. Likewise, topical or systemic corticosteroids may transiently
decrease the mast cell burden and provide symptomatic relief, but they are
associated with long-term cutaneous atrophy, adrenocortical suppression, o
steoporosis, and aseptic necrosis of the femoral head, among other side eff
ects. None of these therapies is directed against a specific cause of masto
cytosis.