spontaneous remission of Burkitt's lymphoma associated with herpes zoster infection

Abstract

A 12-year-old white female with recurrent Burkitt's lymphoma had a spontaneous remission associated with a localized herpes zoster infection. The remission lasted nearly 2 months before the tumor recurred in the central nervous system. LDH isoenzyme determinations done on an earlier ovarian tumor and serum at time of bone marrow relapse showed different predominant LDH isoenzyme patterns. These data might be interpreted as showing that different malignant cell clones were responsible for ovarian and bone marrow relapses. Studies to elucidate the mechanism of spontaneous remission at the time of zoster infection demonstrated serum factor(s) which stimulated normal B lymphocytes.

Case Details

Disease Location

Gi, reproductive organs, cns

Personal Characteristics

12 -year-old female caucasion

Clinical Characteristics

Presented with a several week history of abdominal pain, nausea, and vomiting a barium enema revealed an intussuscepatiention in the terminal ileum with a 3x4cm mass an 18cm span of terminal ileum and 9cm section of cecum were resected, in the middle of which was 4cm tumor the tumor was diagnosed as burkitt's lymphoma invloving the wall of the small intestine, peritoneal fat and the surface of the appendix she was transferred to another hospital ldh values are in figure 1 on the article (not sure how to translate to the spreadsheet) her initial therapy included prednisone, vincristine, cytoxan, intrathecal methotrexate and cns radiation, 2.4k rad; this was followed by escalating doses of methotrexate with citrovorum rescue and cytonxan 10 days later the patient's own bone marrow was harvested in the fifth month post diagnosis, the marrow was free of lymphoma by microscopic exam in the sixth month, the patient discovered abdominal mass and underwent a laparotomy during which the uterus and ovaries were found involved with burkitt's lymphoma she had a radical excision of the entire tumor mass as well as salpingo-oophorectomy and hysterectomy a tumor was also taken off the left abdominal wall the patient was given two doses of adriamycin and continuous cytosine arabinoside over 3 days one month after the ovarian relapse, the patient underwent an autologous bone marrow transplantation after treatment with the crab regimen (the article gets faded and is illegible for a few words, bottom of page 10) after x amount of month(s), she developed herpes zoster along the ophthalmic distribution of the right facial nerve she was started on what later proved to be the placebo arm of an acyclovir trial because suspicious cells were seen in the peripheral smear, a bone marrow exam showed 93% lymphoma cells characteristic of burkitt's lymphoma a trephine biopsy showed that the marrow was hypercellular and nearly replaced by tumor cells no more treatment was decided regarding the relapse, her hemoglobin fell to 8.3gm/dl, wbc count was 1,700/ul. 2 months after her bone marrow relapse (now the 11th month), her ldh rose concomitant with vigorous exercise 1 week later, she developed evidence of a cns relapse with a xii nerve palsy lymphoma cells were seen among the 6 cell/ul in the csf from a lumbar puncture she was started on triple intrathecal therapy of cytosine arabinoside, methotrexate and solucorightef she died a week after the cns resolution from a respiratory arrest (special studies methodology described in-article) the ovarian tumor at first relapse showed a monoclonal b lymphocyte proliferation with mu-kappa specificity, chromosome study of the ovarian tumor revealed an 8:14 translocation

Remission Characteristics

One month after the transplant (8 months from onset), platelets were 197,000/ul, abdominal sonogram showed no masses, bone marrow had decreased megakaryocytes and increased normoblasts with no evidence for tumor, marrow chromosomes were normal, the next week her platelets were 59,000/ul three days after starting the acyclovir trial, skin lesions and virus shedding from the skin decreased by the third day, no virus grew from blood or urine 6 days after the relapse there were no more peripheral blasts, and nucleated red cells which had been up to 4/10 white cells disappeared 18 days after relapse, her hemoglobin had risen to 9.4 gm/dl, wbc count 2,100/ul with no blasts, and platelets were 223,000/ul bone marrow exam one week later showed no evidence of lymphoma by morphologic or cytogenetic analysis she did well over the course of the next 3 weeks with the hemoglobin rising to 10.4gm/dl, wbc to 3,800/ul, and platelets between 159,000-200,000/ul after the three courses of intrathecal therapy, there was no evidence of cns lymphoma and the xii nerve palsy had resolved

Treatment & Mechanisms

Proposed Remission Mechanisms

The zoster infection may have at least induced a potent circulating factor(s) that permitted normal b cells to grow in vitro, the potential for normal b cell growth may have assisted in development of clinical remission it may be that two different clones of b lymphocytes were responsible for her two relapses b cell stimulating activity

Clinical Treatment

Prednisone, vincristine, cytoxan, intrathecal methotrexate, and cns radiation, 2.4k rad, citrovorum rescue excisions, salpingo-oophorectomy and hysterectomy adriamycin and continuous cytosine arabinoside crab regimen and bone marrow transplant triple intrathecal therapy of cytosine arabinoside, methotrexate, and solucorightef

Non-Clinical Treatment

None reported