Regressing Atypical Histiocytosis, A Regressing Cutaneous Phase Of Ki- I - Positive Anaplastic Large Cell Lymphoma
Motley, R. J., Jasani, B., Ford, A. M., Poynton, C. H., Calonje-Daly, J. E., & Holt, P. J. (1992). Regressing atypical histiocytosis, a regressing cutaneous phase of Ki-1-positive anaplastic large cell lymphoma. Immunocytochemical, nucleic acid, and cytogenetic studies of a new case in view of current opinion. Cancer, 70(2), 476–483. https://doi.org/10.1002/1097-0142(19920715)70:2<476::aid-cncr2820700216>3.0.co;2-5
View Original Source →Abstract
The authors believe regressing atypical histiocytosis is a regressing phase of Ki-1-positive anaplastic large cell lymphoma of the skin.
Case Details
Disease Location
Skin (extremities [leg] and trunk)
Personal Characteristics
27 -year-old female patient had a 3 year history of recurrent cutaneous nodules. The lesions enlarged over several weeks and usually ulcerated before spontaneously resolving. Lesions occurred more commonly on the extremities than on the trunk and grew to 1-5cm in diameter. Resolution took from 2-12 months depending on the size of the lesion long history of atopic dermatitis and bronchiectasis
Clinical Characteristics
At the time of examination, new lesions were developing with increasing frequency and she had 3 or 4 nodules at various stages of evolution at any one time cutaneous phase of ki-1-positive alcl on exam, the lesions were firm, indurated, and relatively painless several different forms of local treatment were tried for the cutaneous lesions including curettage, cryotherapy, and surgical excision. Only excision was successful in achieving local remission five years after the clinical onset of the disease, two massive lesions developed on her right leg. They arose over a period of 18 months from small nodules that were clinically and histologocial identical wth the previously remitting lesions radiation therapy was administered hepatomegaly was observed at this time and an additional abdominal CT scan showed multiple deposits in both liver and spleen, confirming the development of extra-cutaneous disease her condition deteriorated rapidly there was initial improvement after chemotherapy with bleomycin and vincristine, but she died a few weeks later materials and methods included in article histology: lesions consisted of a dermal infiltrate, composed mainly of large, polymorphic, highly atypical mononuclear cells. Their cytoplasm was amphophilic or eosinophilic and the nuclei were round, oval, or bean-shaped with coarse granular chromatin and one to several prominent nucleoli. Mitotic figures were frequent and some were highly abnormal. In areas, the infiltrate was predominantly perivascular. Numerous histiocytes and scattered small lymphocytes were seen in the infiltrate. Histologically, condition is characterized by cutaneous nodules composed of large pleomorphic mononuclear cells that express the CD30 epitope. Immunocytochemical analysis: large pleomorphic cells (lpc) expressed the CD30 antigen. Approx. 50% of these cells were found to be ki-67 positive. The lpc virtually all showed cytoplasmic staining for the CD3 antigen. Monoclonal antibodies against the tcrbeta chain and tcrdelta chain showed scattered positivity among small cells in the lesion and dermis, repsectively. Small cells were divided between those that stained with cd45ro, CD5, CD4, and CD68. Some of the t-clymphocytes were also ki-67 positive. Single-cell suspension studies of tumor cells using various monoclonal antibodies and facs analysis showed that there was a population of small lymphoid cells that were CD2+, 8+, and 57+ and a population of larger CD14+. This may reflect lymphocytic and histiocytic infiltration, respectively. Rearrangement data: germine patterns of DNA fragments containing the beta-chain gene were observed in the blood and control tissue after digestion with either of the three enzymes. In the bamhi digest, a 22.6-kilobase ermine fragment was seen in the blood and control tissue, but this had been lost and replace by two faint rearranged bands in the lesion tissue. A faint germine band also was seen occasionally. With ecori digestion, the blood and control showed the germine fragments; the lesion showed loss of the cbeta1 fragments without the appearance of clonal bands (this is imporightant because loss of the cbeta1 fragments only occurs in t-cells). The hindiii digest showed fade out of both germine fragments in the lesion. These results indicate that the DNA studied was derived from polyclonal t-cells. A germine configuration was found in bamhi digests of the lesion using the tcrlambda-chain probe. The lesion contained a germine configuration for the immunoglobulin heavy chain gene. Expression data: functional size messenger RNA coding for tcralpha and beta receptor proteins was found cytogenetic studies: no two cells analyzed showed identical karyotypes, but several common daryotypic features were noticed, including a deletion of the long arm of chromosome 22, additional material in the long arm of chromosome 21, an additional isochromosome for the long arm of chromosome 7, and a derived chromosome 3. Other isolated abnormalities were additional copies of the derived chromosome 3, loss of chromosome 3, abnormalities of the short arm of chromosome 12, the presence of an additional marker chromosome and missing chromosomes 7 and 16.
Remission Characteristics
Dramatic resolution of the two leg lesions occurred after low-dose superficial radiation therapy
Treatment & Mechanisms
Proposed Remission Mechanisms
No major mechanism proposed
Clinical Treatment
Past treatments included curettage, cryotherapy, and surgical excision radiation therapy bleomycin and vincristine (chemo)
Non-Clinical Treatment
None reported