Jones R R, Kobza Black A, Donaghy M, Moshtael O, Pinching A J
Clin Exp Immunol. 1983 Jun;52(3):685-92.
Peripheral blood monocytes from a patient with pyoderma gangrenosum and paraproteinaemia showed defective phagocytosis; longitudinal observations demonstrated an association between the defect, the level of paraprotein and the clinical activity of the pyoderma. Treatment with Melphalan led to a reduction in the paraprotein level and remission of the pyoderma and was accompanied by normalization of monocyte function. After 8 months remission the paraprotein level rose again and was accompanied by a recurrence of the monocyte defect; shortly after this the pyoderma recurred. Pre-incubation of normal monocytes with the patient's plasma or immunoglobulin fractions revealed that a similar defect could be induced in normal cells by the patient's monomeric IgG. The patient's serum also had anti-heparin activity and the relationship between this and the phagocytic defect was explored. These studies indicate a possible pathogenetic mechanism underlying the association between pyoderma gangrenosum and monoclonal gammopathy.
一名患有坏疽性脓皮病和副蛋白血症患者的外周血单核细胞显示吞噬功能缺陷；纵向观察表明该缺陷、副蛋白水平与坏疽性脓皮病的临床活动之间存在关联。美法仑治疗导致副蛋白水平降低，坏疽性脓皮病缓解，并伴有单核细胞功能正常化。缓解8个月后，副蛋白水平再次升高，并伴有单核细胞缺陷复发；此后不久，坏疽性脓皮病复发。用患者血浆或免疫球蛋白组分对正常单核细胞进行预孵育显示，患者的单体IgG可在正常细胞中诱导出类似缺陷。患者血清还具有抗肝素活性，并对其与吞噬缺陷之间的关系进行了探讨。这些研究表明了坏疽性脓皮病与单克隆丙种球蛋白病之间关联的一种可能发病机制。
