Systemic Mast-Cell Disease with

Abstract

NoteMastocytosis is a heterogeneous clinical entity which is classified into four categories: 1-indolent mastocytosis (the most common form), 2-mastocytosis with an associated hematologic disorder, 3-mast cell leukemia and 4-aggressive mastocytosis.

Mastocytosis (urticaria pigmentosa) of skin, stomach, and gut with malabsorption.

“…The histological site of histamine in the stomach (Feldberg and Harris, 1953) indicates the presence of mast cell histamine as well as non-mast cell histamine. Although it has been accepted lately that urticaria pigmentosa is not strictly limited to skin (Berlin, 1955;Reilly, Shintani, and Goodman, 1955;Asboe-Hansen, 1960), and some cases have been reported with gastrointestinal symptoms (Berlin, 1955;Brodeur and Gardner, 1956;Reilly et al, 1955;Zak, Covey, and Snodgrass, 1957), no determinations showing increased gastric and jejunal histamine seem to have been published previously.…”

Urticaria Pigmentosa and Polycythaemia Vera

“…Asboe-Hansen and Kaalund-J0rgensen (1956) described the bone-marrow of a patient with urticaria pigmentosa, systemic mast-cell involvement, and xanthomata, finding it to be maximally hyperplastic and "suggestive of incipient myeloid leukaemia" with myeloid hyperplasia and eosinophilia and with slightly reduced erythropoiesis. Reilly et al (1955) also observe that "other elements of the reticulo-endothelial system" than the mast cell show proliferation in the bone-marrow. Vaughan and Harrison (1939) suggested, as the result of their studies of myelosclerosis following polycythaemia, that there might be an unidentified stimulus to excessive activity of fibroblasts, osteoblasts, haemocytoblasts, and megakaryocytes, all of which are derived from primitive reticulum cells.…”

“…However, enough cases have now been described to confirm that the mast-cell disorders can affect the blood-picture profoundly. Touraine (1939) described a case showing anaemia, leucopenia, and eosinophilia; and Reilly et al (1955) recorded slight anaemia and granulopenia. In Berlin's (1955) case there was a severe progressive macrocytic anaemia, leucopenia, and thrombocytopenia, and many tissue mast cells were demonstrated in the bone-marrow.…”

“…There are, however, many points in which the mast-cell syndromes approach the myeloproliferative disorders. Concerning urticaria pigmentosa, Reilly et al (1955) have stated that "the disorder is best defined as a systemic hyperplasia of one of the cells of the reticuloendothelial system," and there is a similar tendency to involve the spleen, liver, bone-marrow, and lymph nodes.…”

“…With the description by Ellis (1949) of the case of a 1-year-old child, showing associated mast-cell lesions of the liver, spleen, lymph nodes, bonemarrow, and other organs, the systemic manifestations began to emerge. These include splenomegaly and hepatosplenomegaly (Touraine, 1939;Bluefarb and Salk, 1954;Reilly et al, 1955;Brodeur and Gardner, 1956), lymphadenopathy (Little, 1905;Gray, 1938;Degos et al, 1952), and osseous lesions (Sagher et al, 1952;Clyman and Rein, 1952;Sagher et al, 1956). Cases are also described in which systemic mast-cell infiltrations are found in association with skin lesions other than urticaria pigmentosa, and there appears to be a series of distinct but related mast-cell disorders differing mainly in the pattern of organ involvement (Sagher, 1956).…”