Martoia R, Fabresse F, Lagrosse J P, Laissy J P, Timsit J C, Laurens A
Ann Med Interne (Paris). 1979;130(11):537-40.
Malignant histiocytosis, an affection in which there is proliferation of morphologically atypical histiocytes, traditionally associates high fever, deterioration of the general condition, adenopathy, hepatosplenomegaly, and less frequently, cutaneous lesions. Clinical, radiological, and histological signs of bone involvement are rarely observed, which demonstrates the interest of the case reported of a pure medullary form with massive necrosis and successive bone localisations of the osteolytic type. The clinical picture was completed by the progressive development of adenopathy and hepatosplenomegaly. Multiple chemotherapy, according to the A.V.E.C. procedure, controlled the affection for three months before it became totally ineffective. Survival for twelve months after clinical onset demonstrates the extreme malignancy of the affection.
恶性组织细胞增多症是一种形态学上非典型组织细胞增殖的病症,传统上伴有高热、全身状况恶化、淋巴结病、肝脾肿大,较少见的还有皮肤病变。很少观察到骨受累的临床、放射学和组织学征象,这凸显了所报道的一例纯髓质型伴大片坏死及相继溶骨性骨定位病例的价值。淋巴结病和肝脾肿大的进行性发展完善了临床表现。根据A.V.E.C.方案进行的多次化疗在三个月内控制了病情,但随后完全失效。临床发病后存活十二个月表明该病症具有极高的恶性程度。