Lymphoreticular Malignancies Research Articles

Cancer in the Gizan Province of Saudi Arabia: An Eleven Year Study

The occurrence of histologically diagnosed primary malignant tumors in Saudi and Yemeni patients in Gizan Province over the 11 years from 1982 to 1992 was analyzed. During the study period, 1398 malignancies of known primary site were seen in males and 972 in females. In common with other parts of Saudi Arabia, the overall incidence of malignant disease was low by Western standards with lymphoreticular malignancies relatively common and tumors of lung, bowel, breast and the female genital system relatively uncommon. Some malignancies are more common in Gizan Province as compared to elsewhere in Saudi Arabia. Oral cancer was the most common malignancy in females and the most common overall, probably due to widespread use of oral tobacco preparations. Liver cancer was the most common malignancy in males, reflecting the endemic nature of hepatitis B in the area. The incidence of bladder cancer, and particularly of squamous carcinoma of the bladder, was also more, probably due to the high incidence of schistosomiasis in the area. No significant trends in incidence of specific malignancies were noted over the 11-year study period, even though the period has been marked by dramatic and unprecedented changes in lifestyle and public health.

Immunodeficiency Syndromes: X-linked Agammaglobulinemia, Common Variable Immunodeficiency, Chédiak-Higashi Syndrome, Wiskott-Aldrich Syndrome, and X-linked Lymphoproliferative Disorder

The incidence and mortality of neoplasia in patients with primary immunodeficiencies exceed the anticipated rates in the normal population by approximately 100 to 300 times. Lymphoreticular malignancies account for the majority of tumors, although solid tumors, especially gastric carcinoma, occur with increased frequency as well. More than half of these neoplasms are diagnosed by ten years of age, except in patients with a later age of onset of immunodeficiency. Five primary immunodeficiency disorders with an increased risk of neoplasia are reviewed.

Chronic Actinic Dermatitis Is Not Associated with Lymphoreticular Malignancy

Photosensitivity dermatitis/actinic reticuloid syndrome (PD/AR; also called chronic actinic dermatitis) is a chronic photosensitivity disorder that

Use and misuse of biomarker tests in “environmental conditions”

Use and misuse of biomarker tests in “environmental conditions”

Cell-Mediated Immunity in Cancer: Prognostic Significance

DOI: http://dx.doi.org/10.5915/26-2-16366 Cell-mediated immunity (CMI) was studied in 100 patients with cancer and 20 healthy control volunteers with comparable age and sex ratios. Delayed hypersensitivity was estimated by DNCB and PPD cutaneous reactivity in vivo while lymphocyte subpopulations were estimated by peripheral blood lymphocyte (PBL), total rosette forming cells (TRFC) and active rosette forming cells (ARFC) in the peripheral blood. Purified protein derivative (PPD) and Di-nitrochlorobenzene (DNCB) cutaneous reactivity was positive in 4 percent and 64 percent respectively. TRFC values were expressed as a percentage of PBL and were found to be significantly decreased in cancer Stage I (35.5 percent; p< 0.05), Stage II (46.6 percent), Stage III (46.2 percent) and Stage IV (45 percent); the decrease in Stages II, III, and IV was insignificant as compared to the control group (51.5 percent). On the contrary, ARFC levels expressed as a percentage of PBL were significantly decreased in cancer Stage I (21.5%, p< 0.05), but significantly increased in Stage II (35.6 percent), Stage III (33.5 percent) and Stage IV (32.4 percent) as compared to control group (23.6 percent). Since DNCB cutaneous reactivity showed good immunological reserve in advancing stages of cancer in our study, a linear correlation could be established between DNCB cutaneous reactivity and ARFC levels. Since ARFC sub-population is the functionally active T-lymphocyte sub-set against malignant cells in vivo, these cells increased with proliferation of malignant cell mass in vivo, whereas PBL and TRFC levels do not exhibit significant variation. Parameters of cell mediated immunity in vitro TRFC and ARFC were significantly decreased in lymphoreticular and mesenchymal malignancies (p < 0.001). However, ARFC levels were significanlty increased in cancer of gastrointestinal tract (p < 0.05), head and neck (p< 0.05) and breast (p< 0.001) as compared to control group. Linear correlation was found with the percentage of DNCB positive reactors in each group.

Polymorphic reticulosis의 임상적 고찰

Since McBride has described a number of patients with rapid destruction of the nose and face in 1897, many terms were used interchangeably with each other. But polymorphic reticulosis is preferable, because it implies a potentially systemic disease with an unusual lymphoproliferative histopathology of variable atypical lymphoid composition. Polymorphic reticulosis is a relentess, often fatal destructive process of the midface that may progress to involve practically all organ systems. Recently it is considered as a neoplastic process characterized by pleomorphic lymphoreticular cell infiltration pathologically identical to a lymphoreticular malignancy. In this study, I reviewed and evaluated the clical and pathological findings of the PR patients. The results were summarized as follow; The most common age of onset was 4th decade, and male and female ratio was 2.6 to 1. Main symptoms at diagnosis were nasal obstruction, purulent rhinorrhea, and ulceration. The most common site of primary lesion was the nasal cavity and paranasal sinus. There were 17 cases of polymorphic reticulosis, 8 cases of polymorphic reticulosis with features of malignant lymphoma. And progression to a more malignant histiopathologic type was found in 2 cases during follow-up. Main management was radiotherapy with or without chemotherapy. The survival of the group of polymorphic reticulosis with malignant lymphoma was inferior to that of the group of polymorphic reticulosis only. In conclusion, polymorphic reticulosis is more closely linked to lymphoma where transitional forms between the two diseases have been observed. Pathology of atypical cell distribution apperars to be the most important factor in influencing prognosis.

EPSTEIN-BARR-VIRUS HYPERSENSITIVITY OF LYMPHOCYTES FROM PATIENTS WITH ATAXIA-TELANGIECTASIA

Ataxia telangiectasia (AT), an autosomal recessive disorder with a high incidence of lymphoreticular malignancies including Epstein-Barr virus (EBV)-induced lymphoproliferative disorders (LPD), was investigated to assess the susceptibility to EBV infection and oncogenesis. When the patients' lymphocytes were infected with B95-8 EBV, there was a tendency toward an enhanced growth in semisolid agar, as compared with the healthy donor counterparts. Among the preparations tested, from 14 patients, 2 cell lines showed extremely high colony forming efficiency. The lymphocytes from patients with AT did not contain a large number of EBV target cells, as determined by the maximum frequency of EBV-determined nuclear antigen (EBNA) induction prior to cellular DNA synthesis. Fourteen different lymphoblastoid cell lines derived from the 14 patients with AT were then examined for their EBV inducibility and superinfectibility. By treatment with 12-O-tetradecanoyl-phorbol-13-acetate TPA) and culturing at a lower temperature of 33-degrees-C, early antigen (EA) induction occurred approximately 6-fold and 5-fold higher, respectively, as compared with the lymphoblastoid cell lines derived from healthy controls. Viral capsid antigen (VCA) was also induced significantly by TPA or culturing at lower temperature in the lines from patients with AT, but only slightly in the control counterparts. When the lymphoblastoid cells from patients with AT were exposed to P3HR-1 EBV, EA and VCA syntheses were approximately 6- and 12-fold higher, respectively, than those in the cells derived from the healthy controls. This evidence suggested B lymphocytes of patients with AT were highly susceptible to EBV infection and possibly linked to the development of EBV-induced LPD.

Wiskott-Aldrich syndrome: New molecular and biochemical insights

The Wiskott-Aldrich syndrome is an uncommon X-linked recessive disease characterized by eczema, thrombocytopenia, and immunodeficiency. The clinical features begin early in life and include recurrent infections, bleeding, and severe eczema. Unless the condition is treated by bone marrow transplantation, the prognosis of Wiskott-Aldrich syndrome is grave, and premature death caused by sepsis, hemorrhage, or lymphoreticular malignancy is common. Although the biochemical defect responsible for the syndrome is not known, recent investigations with restriction fragment length polymorphisms have mapped the Wiskott-Aldrich syndrome locus to the proximal portion of the short arm of the human X chromosome (Xpl 1 ). The isolation of these DNA markers makes feasible both carrier detection and prenatal diagnosis of Wiskott-Aldrich syndrome and provides an important adjunct to the management of Wiskott-Aldrich syndrome for patients and their families. These genetic data, in conjunction with the recent identification of a specific O-glycosylation defect in lymphocytes from patients with Wiskott-Aldrich syndrome, present an opportunity for the eventual isolation of the Wiskott-Aldrich syndrome gene and identification of the underlying cellular defect. We review the clinical and laboratory features of this syndrome and summarize the new molecular and biochemical approaches that can be used in diagnosis, genetic counseling, and treatment.

Simultaneous measurement, using flow cytometry, of radiosensitivity and defective mitogen response in ataxia telangiectasia and related syndromes.

In a retrospective study, peripheral blood mononuclear cells from 13 patients with known ataxia telangiectasia (AT) (Louis Bar syndrome, McKusick #20890) were irradiated with different doses of X-rays prior to stimulation with phytohaemagglutinin. Mitogen response and cell cycle progression were assessed by two-parameter 5-bromo-2'-deoxyuridine/Hoechst--ethidium bromide flow cytometry. Compared to age-matched controls, AT cells show a severely defective mitogen response in both unirradiated and irradiated cells. Following irradiation with 1.5 Gy, AT cells exhibit significantly greater accumulations of cells in the G2 phase of the first cell cycle than controls. The ratio between the number of cells accumulated in the first cycle G2 phase and the growth fraction provides a clear distinction between AT and control cultures. In addition, two patients with microcephaly, normal intelligence, immunodeficiency, chromosomal instability and risk for lymphoreticular malignancies (Seemanová syndrome) and two patients with the Nijmegen breakage syndrome (both syndromes are listed as McKusick #25126) also exhibit very poor mitogen response and moderately increased G2 phase accumulations after X-irradiation. The simultaneous assessment of radiosensitivity and mitogen response in a single cell kinetic assay provides a speedy and accurate classification of cells of AT and AT-related syndromes.

Lymphomatoid Papulosis

Lymphomatoid papulosis is an elusive and very rare skin disorder. It is clinically benign but histologically "atypical," and about 20% of affected patients go on to develop a lymphoreticular malignancy, usually Hodgkin's disease, or mycosis fungoides. A wide variety of treatments to prevent recurrence and improve local control have been suggested, but the results have been poor. We report on a patient with two nonhealing lymphomatoid papulosis lesions treated successfully with electron beam therapy and describe our technique. The need for close follow-up of all patients with this condition cannot be overemphasized.

Role of circulating immune-complexes as prognostic indicators of lympho-reticular and mesenchymal malignancies in children.

Circulating immune complexes (CIC) were estimated in 28 cases of Non-Hodgkin's lymphomas, Hodgkin's disease, bone and soft tissue sarcomas in the pediatric age group by polyethylene glycol (PEG) precipitation and latex agglutination inhibition (LAI) techniques. Results were compared with 25 age-matched controls. Highly significant CIC values were obtained by LAI technique (P less than 0.01) as compared to PEG pptn technique (P less than 0.05) in malignancy. However, seropositivity for CIC in lymphomas and Hodgkin's disease was 85.71 per cent by LAI test as compared to 57.14 per cent by PEG pptn test. In sarcoma group, seropositivity for CIC was 57.11 per cent by LAI test and 28.57 per cent by PEG pptn test. Combination of both these tests increases the sensitivity of immune complex detection in serum of cancer patients. CIC begin to rise in serum in early stages of neoplastic transformation, and the level of CIC is directly proportional to proliferating tumour mass in vivo.

Drug therapy for inflammatory bowel disease: Part II

Part II of this two-part article (See The American Journal of Surgery 1992; 164: 85-9) reviews the current definition of the role of immunosuppressive therapy in inflammatory bowel disease (IBD) and the use of antibiotics in IBD, as well as summarizes the uses of the new agents on the horizon for the treatment of IBD. Azathioprine and 6-mercaptopurine have steroid-sparing effects in patients with refractory Crohn's disease and ulcerative colitis, treat Crohn's disease-associated fistulas, and are the first agents to demonstrate efficacy in the prophylaxis of Crohn's disease. Their low risk for the development of lymphoreticular malignancy remains a factor in decisions regarding their long-term use. Cyclosporine is steroid sparing in active chronic Crohn's disease and, given intravenously, may help treat severe, refractory ulcerative colitis. Antibiotics have expanding roles: metronidazole is effective for the primary treatment of Crohn's disease, fistulas, abscess, bacterial overgrowth, and pouchitis (after ileoanal anastomosis). Other potential agents show promise in pilot studies but await controlled trials.

Paraproteins in rheumatoid arthritis and related disorders.

There is evidence that the prevalence of monoclonal parparoteinaemia is slightly increased in patients with rheumatoid arthritis. The possibility that this may be a marker of the development of later malignancy in such patients is explored. Mortality rates in rheumatoid arthritis are increased although the development of lymphoreticular malignancy contributes only a small percentage of this increase. However, it does seem likely that patients with longstanding severe rheumatoid arthritis are more at risk of developing myeloma or lymphoma if they have a monoclonal paraprotein band in their serum. IgA paraprotein seems to carry a higher risk than IgG whilst other factors such as urinary free light chains and the presence of secondary Sjögren's syndrome are of less prognostic significance. Similarly a monoclonal paraprotein may identify patients with primary Sjögren's syndrome who have a particular risk of later lymphoma whilst this risk does not appear to extend to patients with systemic lupus erythematosus. Patients with an overlap syndrome do not appear to be at greater risk than those with "pure disease". The association of other rheumatological disease and paraproteinaemia is briefly discussed.

An Unusual Presentation of Secondary Syphilis in a Patient With Human Immunodeficiency Virus Infection

Syphilis has been reported to assume unusual clinical appearances and to exhibit unusual courses in patients infected with the human immunodeficiency virus (HIV) type 1. We recently observed a distinct manifestation of syphilis in an HIV-infected patient with features not previously described.A 38-year-old HIV-seropositive homosexual man presented with fever, chills, malaise, and a cutaneous eruption consisting of indurated, shiny, erythematous plaques that were confluent on the face and scalp leading to alopecia and extreme tautness of the skin. Initial clinical diagnoses included lymphoreticular malignancy and infection. Although cultures yielded Staphylococcus aureus, a skin biopsy specimen was diagnostic of syphilis.This case demonstrates an unusual clinical manifestation of syphilis in a patient with HIV infection and emphasizes the importance of considering cutaneous secondary syphilis in the differential diagnosis of virtually any inflammatory cutaneous disorder in HIV-seropositive individuals.

An unusual presentation of secondary syphilis in a patient with human immunodeficiency virus infection. A case report and review of the literature

Syphilis has been reported to assume unusual clinical appearances and to exhibit unusual courses in patients infected with the human immunodeficiency virus (HIV) type 1. We recently observed a distinct manifestation of syphilis in an HIV-infected patient with features not previously described. A 38-year-old HIV-seropositive homosexual man presented with fever, chills, malaise, and a cutaneous eruption consisting of indurated, shiny, erythematous plaques that were confluent on the face and scalp leading to alopecia and extreme tautness of the skin. Initial clinical diagnoses included lymphoreticular malignancy and infection. Although cultures yielded Staphylococcus aureus, a skin biopsy specimen was diagnostic of syphilis. This case demonstrates an unusual clinical manifestation of syphilis in a patient with HIV infection and emphasizes the importance of considering cutaneous secondary syphilis in the differential diagnosis of virtually any inflammatory cutaneous disorder in HIV-seropositive individuals.

Familial microcephaly with normal intelligence in a patient with acute lymphoblastic leukemia

The authors describe a family with two children with microcephaly and normal intelligence, in which acute lymphoblastic leukemia developed in one of the siblings. An autosomal recessive pattern of inheritance is suggested by the pedigree. This is consistent with the literature, which the authors reviewed. All of the patients have similar phenotypic features, with some demonstrating chromosomal instability. It is important to recognize this syndrome because of the increased risk of lymphoreticular malignancy.

Cutaneous lesions of hemophagocytic syndrome in a patient with T-cell lymphoma and active Epstein-Barr infection

The hemophagocytic syndrome (HPS) was first described in immune-compromised patients with secondary viral infections. Immunodeficiency has not always been diagnosed before the onset of HPS, but most patients who develop HPS have known immune deficits. HPS has also been reported in association with lymphoreticular malignancies. In HPS the most prominent histologic change is phagocytosis of red blood cells and other bone marrow-derived elements by cytologically benign histiocytes. Considerable confusion exists about the similarities and differences between HPS and other conditions in which hemophagocytosis may occur, for example, histiocytic medullary reticulosis, T-gamma lymphoma, malignant histiocytic lymphoma, and cytophagic histiocytic panniculitis. We report a patient with HPS and cutaneous lesions of cytophagic histiocytic panniculitis who also had an active Epstein-Barr virus infection, and a CD8+ T-cell lymphoma.

Early Bone Marrow Transplantation in an Infant with Wiskott-Aldrich Syndrome

The Wiskott-Aldrich Syndrome (WAS) is a rare X-linked immunohematological disorder characterized by eczema, profound thrombocytopenia, and progressive immunodeficiency. Severe hemorrhage, overwhelming sepsis, or lymphoreticular malignancy usually cause death in childhood. Recently, bone marrow transplantation (BMT) has been curative in some well-established cases, but there is no general agreement about the place of BMT in infants with WAS before the development of significant immunological abnormalities. We describe the successful use of early histocompatible BMT in a 10-month-old infant in whom WAS was diagnosed on the basis of eczema, thrombocytopenia, small platelets, and raised serum immunoglobulin A (Ig) and IgE, but before the development of immunodeficiency as evidenced clinically by recurrent infections, or immunologically by low serum IgM or consistently abnormal lymphocyte responses to mitogens. After an unstable period for several weeks posttransplantation when he developed marked hepatomegaly and severe interstitial pneumonitis, he made a good recovery. His eczema and thrombocytopenia resolved and he has shown no clinical or laboratory evidence of immunodeficiency. It is now over 2 years since his BMT. Because of the poor prognosis of WAS, where a histocompatible donor is available, BMT at the earliest opportunity, despite the inherent risks of such a procedure, may be the best option for an infant with WAS.

Autoimmunity and malignancy

In patients with autoimmune diseases malignancy is observed more frequently than in an age-matched control group. In strains of mice susceptible to autoimmune diseases, the incidence of lymphoreticular malignancies is increased. Although viruses have not been directly implicated in their pathogenesis or etiology, there are suggestions that slow viruses may be involved. Viruses have been found to be important in the development of lymphomas. Hence in genetically susceptible animals, viruses can initiate the autoimmune process. Prolonged stimulation of the autoimmune process or a prolonged state of immunodeficiency could trigger or activate genes or their products, which could result in the development of malignancy.

Cutaneous Presentation of Acute Myeloid Leukemia in a « Classical » Kaposi's Sarcoma Patient

A case of early cutaneous involvement by an acute myeloid leukemia in a Caucasian man, previously affected by a "classical" Kaposi's sarcoma is reported. The diagnoses were based upon histologic examinations and appropriate hematologic investigations. After reviewing extensively the literature about the association of lymphoreticular system malignancies and Kaposi's sarcoma, possible implications and hypothetical etiopathogenetic mechanisms are discussed.