Psychosocial symptomatology, personal growth, and development among young adult patients following the diagnosis of leukemia or lymphoma.

Leukemias and lymphomas, especially Hodgkin's disease, are common cancers in young adults. Young adulthood is also a critical period for psychological and social development. The occurrence of cancer can interfere with the development of independence, self-image, and life goals of young adult patients. Young adult patients with leukemia or lymphoma, especially those with less favorable prognoses, experience areas of significant personal growth and maturation during their illness and treatment. Close family and social supports report as much psychosocial stress, and in many cases more stress, than the patients themselves, but similar patterns of personal growth are rarely seen.

Sequential hydroxyurea-cytarabine chemotherapy for refractory non-Hodgkin's lymphoma.

In experimental systems, hydroxyurea (HU) and cytarabine (ara-C) produce synergistic cytotoxicity to murine and human leukemia cells due to both cytokinetic and biochemical interactions that tend to enhance the effectiveness of ara-C. Therefore, we began a phase II trial of the combination of HU and ara-C to determine the efficacy and toxicity of this combination in treatment of patients with refractory non-Hodgkin's lymphoma. Chemotherapy began with HU 500 mg administered orally every six hours for four doses. Twelve hours following the fourth HU dose, ara-C 100 mg/m2/d was administered by continuous intravenous (IV) infusion for three days. Concomitantly with the three-day ara-C infusion, patients again received HU 500 mg orally every four hours. Cycles of therapy were repeated every 28 days. Twenty-five patients ranging in age from 26 to 70 years were enrolled in the study. Of 21 patients evaluable for response, nine (43%) obtained complete (CR) or partial remissions (PR). Most responding patients had either large-cell or cutaneous T cell lymphoma, and all but two had a performance status of 0 to 1 at entry in the study. The median survival for all responding patients was 13 months compared with 2.5 months for nonresponders. Patients obtaining a CR had a median survival of 27.5 months, and two of the four CRs remain alive and in remission at 10+ and 30+ months from achievement of CR status. The primary toxic effect of this regimen was bone marrow suppression. The median WBC nadir was 2,200 cells/microL, and the median platelet nadir was 80,000/microL. Other toxicities included mild nausea and vomiting and diffuse maculopapular rash. This biochemically rational approach to enhancing ara-C activity may have significant clinical utility and should be further explored in treatment of patients with large-cell and cutaneous T cell lymphomas.

Treatment of diffuse histiocytic lymphoma (DHL) with COMLA (cyclophosphamide, oncovin, methotrexate, leucovorin, cytosine arabinoside): a 10-year experience in a single institution.

Between March 1974 and December 1983, 83 patients with diffuse histiocytic lymphoma (DHL) were treated with COMLA (cyclophosphamide 1.5 g/m2 day 1; Oncovin (Lilly, Indianapolis) 1.4 mg/m2 days 1, 8, and 15; and cytosine arabinoside 300 mg/m2 and methotrexate 120 mg/m2 days 22, 29, 36, 43, 50, 57, 64, and 71; and leucovorin 25 mg/m2 every six hours X 4, beginning 24 hours after methotrexate). For the purpose of analysis, patients were divided into two groups. Group 1 (n = 54) included patients age 65 or under who had received no prior curative radiotherapy or chemotherapy. Group 2 (n = 29) included all patients over age 65 and patients who had received prior curative radiation therapy or prior minimal chemotherapy. The median time of follow-up for all patients was 28 months. Group 1 included 11 stage II, ten stage III, and 33 stage IV patients. Of 48 evaluable patients in this group, 21 (44%) achieved a complete remission (CR), eight (17%) achieved a partial remission (PR), and 19 (40%) showed no response (NR). Median survival of CR patients was 114+ months, PR patients, 42 months, and NR patients, 13 months. Six CR patients relapsed. The median disease-free survival of CR patients was 108+ months. Group 2 included nine stage II, seven stage III, and 13 stage IV patients. Of 24 patients evaluable for response, eight (33%) achieved a CR, six (25%) achieved a PR, and ten (42%) showed no response. The median survival of CR patients was 114+ months, that of PR patients was 17 months, and that of NR patients, 9 months. Two CR patients relapsed. The median disease-free survival of CR patients had not been reached at 102 months. The regimen was well tolerated in most patients and toxicity was acceptable. We conclude that COMLA is a well tolerated outpatient chemotherapy regimen capable of inducing durable CRs in some patients with DHL. Results achieved with COMLA, however, are inferior to those of more aggressive treatment programs; thus, the use of COMLA as first-line therapy in DHL should be limited to those patients unable to tolerate a more aggressive treatment program.

Long-term survival of patients with localized diffuse histiocytic lymphoma.

From January 1970 to March 1981, localized diffuse histiocytic lymphoma (DHL) was identified in 31 patients by exploratory laparotomy and splenectomy (pathologic stage I, 17 patients; pathologic stage II, 14 patients) at the University of Chicago. The median follow-up time was 72 months. All patients were previously untreated and received radiation therapy as their primary treatment modality. Chemotherapy was administered only at the time of relapse. All but two patients achieved a complete remission (CR) with radiation therapy. The actuarial disease-free survival for patients with stage I disease is 94% at 5 years and 72% at 10 years. For stage II disease, the disease-free survival is 56% at 5 years and 31% at 10 years. The difference in the disease-free survival between stage I and II is statistically significant (P = .02). The survival at 10 years is 70% for stage I disease and 46% for stage II disease. Five patients had documented relapses (four had stage II disease). Only two of those who relapsed achieved a second CR with salvage chemotherapy. Our data show an excellent outcome in patients with pathologic stage I disease, indicating that a high percentage of these cases can be cured with radiotherapy alone. Patients with clinical stage II disease might be served better with chemotherapy.

Pathologic stages IA and IIA Hodgkin's disease: results of treatment with radiotherapy alone (1968-1980).

Between Jan 1, 1968, and Dec 31, 1980, 108 previously untreated patients with Hodgkin's disease pathologic stages (PSs) IA (29 patients) and IIA (79 patients) initially received radiotherapy alone. One postoperative death (due to pulmonary embolus) (0.9%) occurred, with one serious complication (0.9%). Between 1968 and 1973, patients were randomized to receive either involved field radiation treatment (RTIF) or extended field radiation treatment (RTEF). Since 1973 all patients have received RTEF, 4,000 cGy in four to five weeks, with a median follow-up of 7.4 years. Complete remission (CR) was achieved in 102 patients (94.4%), with no significant difference according to treatment or stage. Of the complete responders, 25 patients relapsed: 5/15 RTIF and 20/87 RTEF (P = .6). Twenty-one of 25 relapsing patients achieved a second CR. Disease free survival rates at five and ten years constituted: PS IA, 78.6% for both; PS IIA, 74.8% and 73.1% (P = .6); RTEF, 76.7% for both; RTIF, 73.3% and 66.7% (P = .7). Eighteen patients have died: eight of recurrent lymphoma, two of pulmonary embolus, one each of myocardial infarction, pulmonary fibrosis, and acute nonlymphocytic leukemia (ANLL) (following salvage chemotherapy), and one of diffuse histiocytic lymphoma (DHL). Four patients died in remission of unrelated causes. Actuarial survival rates at five and ten years constituted: PS IA, 95.7% and 72.4%; PS IIA, 89.6% and 81.4% (P = .3); RTIF, 93.7% for both; RTEF, 90.7% and 71.2% (P = .2). Age, sex, number of sites, and mediastinal involvement did not influence the outcome. Acute toxicity was modest and more frequent among those receiving RTEF (P = .08). Chronic toxicity (onset more than 30 days after completion of treatment) was identified in 16 patients: 1/16 RTIF; 15/92 RTEF (P = .5). Three patients developed a second malignancy: one carcinoma of the cervix in situ; one ANLL (following salvage chemotherapy); and one DHL of the stomach. At least 75% of patients with PS IA and IIA Hodgkin's disease were cured by radiation alone, with a risk of secondary malignancy following radiation alone of 0.9%. Since the majority of relapsing patients were successfully salvaged by chemotherapy, radiation alone appears to be the initial treatment of choice in this group of patients.

Long-term survival of patients with intermediate and high grade lymphoma treated with COMLA/ABP.

Between September 1983 and April 1985, 15 patients with previously untreated intermediate and high grade lymphoma were treated with COMLA/ABP (cyclophosphamide, vincristine, cytarabine, methotrexate, leucovorin, adriamycin, bleomycin, prednisone). There were nine males and six females; age ranged between 36 and 77 years (median, 58). Histologic diagnoses included five patients with diffuse large cell, six patients with immunoblastic, three patients with composite, and one patient with follicular large cell with diffuse areas. Following therapy, 10 patients (67%; 95% confidence interval (Cl), 43-91%) achieved a complete remission (CR) including five of six patients with immunoblastic; three patients (20%) had a partial remission, and two patients (14%) had no response. Toxicity was acceptable. After a minimum follow up of 4 years, there has been only one relapse which occurred 7 months after CR. The 4-year survival for all 15 patients is 65% (95% Cl, 40-89%). The COMLA/APB program produced a high rate of complete and durable remissions especially among patients with immunoblastic lymphoma.

Aggressive large cell lymphoma of the ileum after long-term cyclophosphamide therapy for breast carcinoma.

A middle-aged woman was treated for breast carcinoma with postoperative adjuvant chest wall irradiation, followed four and seven years later with therapy to spinal ports for palliation of metastatic disease. For the next three and a half years, she received oral cyclophosphamide on a daily basis to a total of more than 110 g. Twelve years after diagnosis and five years after the start of chemotherapy, an aggressive, large cell lymphoma of the ileum developed, with poor response to conventional therapy. This may represent the first patient with breast carcinoma in whom a treatment-induced non-Hodgkin's lymphoma has developed.

Neurocrest and colonic tumors: new clinical syndrome. Report of three cases.

This report describes three patients with both multiple intestinal polyps and tumors of neural crest origin. This combination of findings may represent a new clinical syndrome. The embryologic relationships between tumors derived from endoderm and tumors derived from neurocrest are described. An inherent defect in tissue proliferation or repair is postulated to explain the abnormal growth in these two different cell lines.

Chromosomal aneuploidy in a patient with hypereosinophilic syndrome. Evidence for a malignant disease.

A patient with rapidly fatal hypereosinophilic syndrome and a bone marrow chromosomal abnormality, 49,XYY,t(3:5),+8,+mar, is described. Scanning and transmission electron microscopy of eosinophils failed to reveal any significant abnormalities. Previous cytogenetic data on patients with hypereosinophilic syndrome have been deficient in that few studies have been performed with banding technics. These technics may help in the classification of hypereosinophilic syndrome and predict which patients will have a rapidly fatal course.

The patterns of failure in patients with pathological stage I and II diffuse histiocytic lymphoma treated with radiation therapy alone.

Radiation therapy was used to treat 36 patients with pathological Stage I and II diffuse histiocytic lymphoma at The University of Chicago Hospitals from 1970 to 1986. Twenty-two patients had pathological Stage I and 14 had pathological Stage II diffuse histiocytic lymphoma. The patients were treated with a median tumor dose of 50 Gy (range of 40-60 Gy). Therapy consisted of extended field radiation therapy in 27 patients (extended mantle or total nodal irradiation) and involved field irradiation in nine patients. The 10-year actuarial relapse-free survival for pathological Stage I and pathological Stage II patients was 91% and 35%, respectively (median follow-up of 7 years). None of the 22 pathological Stage I patients had bulky mediastinal or abdominal disease. Of the 22 pathological Stage I patients, one failed in an unirradiated contiguous lymph node and one relapsed with disseminated disease. Of the 14 pathological Stage II patients, two patients with bulky disease failed in field, one patient failed in a contiguous node, three patients failed within the abdomen, and three patients failed with disseminated disease. To better evaluate the efficacy of staging laparotomy, we analyzed the patterns of failure of 17 clinical Stage I and II diffuse histiocytic lymphoma patients. Four of these patients failed in field (three in sites of bulky disease), and five patients relapsed in the abdomen (three with disseminated disease). Salvage treatment with multiagent chemotherapy resulted in second complete responses in seven of ten patients; however, all but one have recurred and are dead of disease. Radiation therapy may be used as the sole treatment in patients with pathological Stage I diffuse histiocytic lymphoma without bulky disease. Patients with pathological Stage II diffuse histiocytic lymphoma and clinically staged patients have a higher incidence of dissemination and relapse within the abdomen. A benefit resulting from the administration of extended field irradiation was not revealed by this study.

Radiation therapy for pathologic stage III Hodgkin's disease with and without chemotherapy.

Ninety-eight patients with pathological Stage (PS) III Hodgkin's disease treated between 1969 and 1984 were retrospectively analyzed. Treatment consisted of radiation therapy (RT) alone in 46 patients and combined radiation therapy and chemotherapy (CMT) in 52 patients. The median follow-up was 10 years (range 3-19 years). Fifteen-year year survival for patients with Stage III1-is better than for Stage III2 patients (82% vs 53%; p = .014). Patients with Stage III1A have a favorable prognosis regardless of treatment modality. The probability of freedom from relapse at 15 years for patients with pathological Stage III1A treated with radiation therapy is 70%, compared to 83% for pathological Stage III1A patients treated with combined modality therapy (p = .56). In patients with pathological Stage III2A, III1B, and III2B relapses were less frequent with the use of combined modality therapy compared to radiation therapy. We conclude that pathological Stage III1A patients may be treated with radiation therapy alone; the other subsets of patients benefit from combined radiation and chemotherapy.

Malignancies of undetermined primary origin.

Unknown primary malignancy (UPM) is not a disease entity. Rather, it represents a variety of different metastatic, malignant neoplasms all presenting with either an occult primary or having such a highly undifferentiated histologic appearance that an accurate pathologic classification on routine hematoxylin-eosin section is not possible. UPM is a spectrum of malignancies that includes those that are treatable and curable and those for which no specific treatment exists. For the physician, a diagnosis of UPM represents a beginning rather than an end. The minimal workup of such patients includes a thorough history and physical examination, complete blood counts, urine analysis, multichannel chemistries, a chest radiograph, and computed tomography of the abdomen and pelvis. Having completed this workup, further tests are unnecessary and unwarranted unless specific symptoms or physical signs exist. Once the aforementioned workup is completed, the physician must communicate frequently and freely with the pathologist as further diagnostic tests will be laboratory based and include electron microscopy, histochemical stains, and immunocytochemistries. Immunocytochemistries are relatively new laboratory procedures which have made a significant contribution in the accurate pathologic diagnosis of a tissue specimen that in years past would have been classified as an unidentified malignant neoplasm. An initial panel of immunocytochemistries (vimentin, cytokeratin, CEA, and common leukocyte antigen) should be performed on the tissue block in patients with UPM as they provide direction in the accurate classification of the malignant neoplasm. Chromosomal analysis of tissue is useful in the recognition of lymphomas or soft-tissue sarcomas which would otherwise be classified as UPM. In years to come, when specific DNA probes capable of identifying specific chromosomal rearrangeaments are widely available, pathologic classification of UPM will be performed on a molecular level. Some unknown primary malignancies are treatable and potentially curable. These include large cell lymphoma, extragonadal germ cell malignancies, squamous cell carcinoma metastatic to cervical lymph nodes without an obvious primary, metastatic adenocarcinoma to axillary lymph nodes in women (invariably on occult breast primary), and malignant ascites in women, which usually represents ovarian cancer. Metastatic adenocarcinoma of unknown primary origin, with the exception noted above and the rare presentation of an occult prostate cancer as UPM, is an ultimately fatal malignancy with a relatively shor clinical course.(ABSTRACT TRUNCATED AT 400 WORDS)

An approach to general oncology: principles and procedures.

The obstetrician-gynecologist is frequently the only physician to attend women during their reproductive years. Malignant disease outside the pelvic organs is a fairly frequent occurence in this age group. Certain findings in the patient's history and physical examination can suggest malignant disease. The routine laboratory examination can also provide indications of the presence of a neoplastic process. Once this process is suspected, histologic proof of malignancy must be obtained before further staging and therapy are considered. The extent of the disease is important for the planning of therapy, i.e., whether it is to be surgery, radiotherapy, chemotherapy or some combination of the three. Adjuvant chemotherapy is recognized as a form of treatment designed to eradicate micrometastases, prevent the occurrence of subsequent clinical metastatic disease and, as a result, improve survival.

Hemolytic anemias during pregnancy and the reproductive years.

Anemia is a common phenomenon in women during the reproductive years. In pregnancy, it is associated with an increased incidence of maternal-fetal morbidity and mortality. The approach to the investigation of anemic women suspected of having hemolytic anemia of either congenital or acquired etiology is the subject of this article. Various conditions in the pregnant women can have hematologic consequences for the newborn infant; these conditions include sensitization to fetal blood cells, infections, drug ingestion and the possession of genes for hereditary hemolytic disorders, which may be transmitted to the fetus. Because several forms of hemolytic anemias are hereditary or are caused by an altered gene, genetic consultation is important.