Urticaria Pigmentosa and Acute Lymphoblastic Leukemia

Fromer, John L.

doi:10.1001/archderm.1973.01620170091026

Cited by 24 publications

(7 citation statements)

References 7 publications

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“…24 Paradoxically, cutaneous mastocytosis lesions may at this point fade or even disappear. 40 Only a few cases of aggressive mastocytosis, also known as lymphadenopathic mastocytosis with eosinophilia and classified as category III of mastocytosis (Table 1), have been reported in the literature. 24 As therapeutic and prognostic implications may be different, it is important to distinguish between these two conditions.…”

Section: Systemic Manifestationsmentioning

confidence: 99%

“…19 Compared with other haematological disorders, lymphocytic leukaemia appears to be more often associated with mastocytosis in children. 40 Only a few cases of aggressive mastocytosis, also known as lymphadenopathic mastocytosis with eosinophilia and classified as category III of mastocytosis (Table 1), have been reported in the literature. 9 Mast cell leukaemia, corresponding to category IV, is an extremely rare disease which presents with atypical immature mast cells that diffusely infiltrate the bone marrow and that account for more than 10% of blood cells.…”

Section: Systemic Manifestationsmentioning

confidence: 99%

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Mastocytosis: recent advances in defining the disease

2001

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Mastocytosis is a rare disease characterized by a primary pathological increase in mast cells in different tissues, which may present in a variety of clinical patterns. Major advances have been made in recent years in the understanding of the pathogenesis of mastocytosis. This review is aimed at familiarizing dermatologists with these recent findings, and at exploring their possible implications for the diagnosis and treatment of the condition. The heterogeneous clinical presentation of mastocytosis is detailed with respect to the type of skin lesions, age at onset, family history, organ systems involved, associated haematological disorders and prognosis. Recent genetic findings also indicate different pathogenetic forms of mastocytosis, as adult patients and those with associated haematological diseases usually express activating mutations of the stem cell factor receptor c-kit, whereas most cases of childhood-onset and familial mastocytosis seem to lack these mutations. Despite the presence of c-kit mutations, patients with cutaneous lesions generally have a good prognosis, even when there is involvement of other organs. Some patients, particularly those with childhood-onset disease, experience spontaneous remission, mostly by puberty. c-kit mutations do not explain the initial cause of mastocytosis, and their prognostic significance is as yet unclarified, as is the pathogenesis in patients without the mutations. Furthermore, these novel findings have as yet not resulted in a more effective treatment of the cause of the disease, so that counselling, prevention of exposure to mast cell secretory stimuli, and symptomatic treatment remain the mainstays of current patient management.

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Section: Systemic Manifestationsmentioning

confidence: 99%

Section: Systemic Manifestationsmentioning

confidence: 99%

Mastocytosis: recent advances in defining the disease

2001

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“…Several reports of the occurrence of acute lymphocytic leukaemia in rapidly progressive and late onset paediatric onset mastocytosis suggest the possibility of a predilection for the development of a haematological malignancy in these patients. 71 In summary, paediatric onset mastocytosis is an unusual disease with an often benign course. The disease in children is less likely to have a systemic component.…”

Section: Prognosismentioning

confidence: 99%

Paediatric mastocytosis

Carter

2002

Archives of Disease in Childhood

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“…Cutaneous expressions of mastocytosis are considered to be benign and indolent but haematological malignancy is a recognized complication 6–8 . There have been estimates of the frequency of systemic manifestations and bone marrow involvement in patients with UP 9–15 …”

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confidence: 99%