Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In the management of photosensitivity dermatitis/actinic reticuloid syndrome (PD/AR) (syn. chronic actinic dermatitis), a chronic and often severe photodermatosis, there has been concern that patients may be predisposed to the development of lymphoreticular malignancy. A follow-up study of 231 patients with PD/AR who had been investigated at the Photobiology Unit, Ninewells Hospital, Dundee, between 1971 and 1991, was undertaken to determine (i) the incidence and type of malignancies (ii) the causes of any deaths. This information was obtained from three sources: (a) National Cancer Registry data, (b) death certificates, (c) when possible or practical, by casenote review. Thirty-eight malignancies had occurred (in 37 of the 231 patients). Although six of the 38 malignancies were lymphoma registrations, it emerged from a review of casenotes, pathology reports and death certificates that five of the six were incorrect: two were labelled 'mycosis fungoides' prior to diagnosis of PD/AR; a case of dermatopathic lymphadenopathy was initially reported as Hodgkin's disease; clerical errors had occurred in two cases. The remaining case was a true B-cell lymphoma. The occurrence of one lymphoreticular malignancy is not significantly different from the number expected in a normal population (0.96), when applying 5-year age-, sex-, and site-specific incidence rates to the cumulative patient years of risk [standardized incidence ratio 1.04 (95% CI 0.03-5.79)]. There was also no significant increase in the risk of non-lymphoma malignancies in the PD/AR subjects. Since diagnosis, 83 patients have died, the majority from cardiorespiratory or cerebrovascular diseases, or the reported malignancies, a pattern expected in an elderly population.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The photosensitivity dermatitis and actinic reticuloid syndrome: no association with lymphoreticular malignancy. 791 84

Omenn syndrome is a variant of SCID, inherited as an autosomal recessive disorder, and characterized by severe eczematoid dermatitis, eosinophilia, elevated serum IgE and a distinctive histology in enlarged lymph nodes. The etiology of Omenn syndrome is unknown, however, unlike other forms of SCID; patients with Omenn syndrome have activated T lymphocytes in their circulation capable of non-MHC restricted cytotoxic function. Recently, it has been observed that the use of immunosuppressive therapy, particularly cyclosporine, can modify the clinical manifestations of the disorder. Prior to the use of bone marrow transplantation this disease was universally fatal. Death typically occurred in infancy as the result of opportunistic infections and/or malignancies, most notably lymphomas. While bone marrow transplantation has become quite successful for many phenotypes of SCID, even with the use of alternative donors other than histocompatible siblings, in Omenn syndrome it remains a challenge. In our experience, patients with Omenn syndrome exhibit a higher incidence of Gram negative sepsis, before and during transplantation, and carry a significant risk of post-transplant rejection when compared with patients with other phenotypes of SCID. We report the results of six patients treated with bone marrow transplantation from alternative donors, three had unrelated donors (URD) and three had haplo-identical parental donors. Five of the six patients achieved complete and/or durable donor cell engraftment and only one patient experienced acute GVHD. Three patients died of transplant-related complications (infection or EBV-associated B cell lymphoma) between day +22 and day +95 post-transplant. Three patients survived more than 1 year post-transplant.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Mismatched bone marrow transplantation for Omenn syndrome: a variant of severe combined immunodeficiency. 853 10

A seven-year-old neutered male cocker spaniel was presented with an 11-month history of generalised bacterial dermatitis. There were skin lesions over the entire body, which were round, slightly raised and encrusted. Skin biopsies were collected and the histological findings were consistent with pemphigus foliaceus. Immunohistochemical staining by the indirect immunoperoxidase method was positive, with desmosomal deposition of immunoglobulin (Ig) G. Haematological analysis revealed a regenerative anaemia and profound thrombocytopenia, while a Coombs' test was positive for polyvalent canine Coombs' reagent and anti-dog IgG. An antinuclear antibody test was positive, with a titre of 10,240. An ophthalmic examination demonstrated low tear production (keratoconjunctivitis sicca). Seven months after initial referral, the dog was re-presented with severe generalised peripheral lymphadenopathy. Radiographic evaluation of the thorax and abdomen revealed enlarged cranial mediastinal and sublumbar lymph nodes. Tru-Cut biopsy from an enlarged lymph node confirmed the diagnosis of lymphoma, which was phenotyped as a B-cell tumour. The diagnosis in this case was systemic lupus erythematosus, with the unusual feature of pemphigus foliaceus, and subsequent development of B-cell lymphoma. The case adds further to knowledge of the protean clinical presentations of canine autoimmune diseases and provides additional evidence for the potential association between autoimmunity and immune-system neoplasia in this species.
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PMID:Pemphigus foliaceus in association with systemic lupus erythematosus, and subsequent lymphoma in a cocker spaniel. 1087 6

A 50-year-old patient, suffering from familial polyposis (Gardner's syndrome), initially presented with several nodules on his left arm. Histological examination revealed primary cutaneous T-cell-rich B-cell lymphoma (TCRBCL). Staging procedures failed to detect any systemic involvement. Three years after total excision of the tumours, the patient presented with a non-specific dermatitis, enlarged axillary lymph nodes and splenomegaly. Histological and immunohistochemical examination of lymph node and spleen biopsy specimens resulted in the diagnosis of Hodgkin's disease (HD) of the nodular sclerosis type. Sequence analysis of single cells micromanipulated from skin and from lymph node lesions indicated that both lymphoma infiltrates were derived from the same precursor germinal centre B-cell clone. This is a case showing a clonal relationship between TCRBCL and HD, providing support to the B-cell origin of Hodgkin and Reed-Sternberg cells.
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PMID:Primary cutaneous T-cell-rich B-cell lymphoma and Hodgkin's disease in a patient with Gardner's syndrome. 1114 53

The presence of a granulomatous reaction in lesions of cutaneous lymphomas has been described in the past in several cases. Especially in mycosis fungoides, a "granulomatous" variant of the disease has been well characterized. We studied the clinicopathologic features of cutaneous lymphomas with prominent granulomatous reaction, including both cutaneous T-cell lymphomas and B-cell lymphomas (primary cutaneous lymphoma 22, secondary cutaneous lymphoma one). Biopsies of 23 patients with histopathologic features of cutaneous T-cell lymphoma or cutaneous B-cell lymphoma with prominent granulomatous reaction were included in this study. A prominent granulomatous reaction was defined as the presence of a granulomatous component exceeding 25% of the dermal infiltrate. There were 14 cases of mycosis fungoides, two of subcutaneous panniculitis-like T-cell lymphoma, four of small/medium pleomorphic T-cell lymphoma, one of follicle center cell lymphoma, one of large B-cell lymphoma, and one of secondary cutaneous peripheral T-cell lymphoma. Altogether, a prominent granulomatous reaction could be observed in 1.8% of all patients with cutaneous lymphoma (primary or secondary) registered in the files of the Department of Dermatology of the University of Graz (Graz, Austria), demonstrating that there is a distinct, albeit small, proportion of cases revealing this peculiar reaction pattern. In seven cases a misdiagnosis of granulomatous dermatitis preceded the correct diagnosis for a period of 1-216 months, suggesting that sequential biopsies and complete phenotypic and molecular genetic analyses should be carried out in cases of "unusual" granulomatous dermatitis.
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PMID:Cutaneous lymphomas with prominent granulomatous reaction: a potential pitfall in the histopathologic diagnosis of cutaneous T- and B-cell lymphomas. 1271 56

Atopic dermatitis is a chronic relapsing skin disease that occurs commonly during early infancy and childhood and is frequently associated to personal and family history of allergic rhinitis, asthma and/or skin disease. As a rule, it follows a benign course. We report a case of a female patient with a chronic atopic-like dermatitis who eventually developed an extranodal systemic large B-cell lymphoma during the course of her disease.
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PMID:Extranodal B-cell lymphoma in a patient with atopic-like dermatitis. 1503 28

The rituximab antibody is a genetically engineered chimeric murine/human monoclonal antibody directed against the CD20 antigen found on the surface of normal and malignant B lymphocytes. Rituximab is indicated for the treatment of patients with relapsed or refractory, low-grade or follicular, CD20-positive, B-cell non-Hodgkin lymphoma. Rituximab is also commonly used to treat chronic lymphocytic leukemia, Waldenstrom's macroglobulinemia, and immune or idiopathic thrombocytopenic purpura (ITP). Rituximab is an effective treatment for primary cutaneous B-cell lymphoma and other cutaneous lymphomas. Rituximab is an effective treatment for mixed cryoglobulinemia. Rituximab is a promising treatment for systemic lupus erythematosus, dermatomyositis, pemphigus, vasculitis, and a variety of hematologic diseases. Black-box warnings on rituximab include fatal infusion reactions, tumor lysis syndrome, and severe mucocutaneous reactions. A variety of cardiac, pulmonary, renal, and hematologic side effects can occur. It commonly causes mild cutaneous side effect and rarely has caused paraneoplastic pemphigus, Stevens-Johnson syndrome, lichenoid dermatitis, vesiculobullous dermatitis, and toxic epidermal necrolysis.
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PMID:A review of rituximab in cutaneous medicine. 1663 71

Lyme borreliosis is a tick-borne zoonosis due to bacterial infection by Borrelia (B.) burgdorferi sensu lato The disease presents differently in Europe or North America and may be called European borreliosis when acquired in Europe. Lyme borreliosis evolves in 3 stages. The main manifestations include cutaneous, neurological, and joint involvement. Erythema migrans (EM) is the most specific and most frequent finding in patients with Lyme borreliosis. It is the hallmark of early-localized borreliosis. EM is a slowly expanding red macula that occurs in about 60-80% of patients contracting Lyme borreliosis. Central clearing of the red patch can occur. It appears at the site of the tick bite, 7 to 20 days after the bite. Borrelial lymphocytoma (BL) occur rarely in patients with the early-disseminated stage of the disease. BL is a red or brown nodule or plaque located on the nipple, the earlobe, the scrotum, or the face. It should not be confused with cutaneous B-cell lymphoma. Acrodermatitis chronica atrophicans (ACA) is the cutaneous manifestation of late borreliosis. It starts as a violaceous patch, usually located on the extensor surface of a limb. Periarticular nodules and cords can also be present. Without treatment, it will evolve over weeks or months to the typical atrophic stage with extensive dermo-epidermal atrophy and visibility of superficial veins. Only these 3 manifestations are clearly related to an infection with B. burgdorferi. The relationship between infection with B. burgdorferi and other dermatoses, especially morphea, lichen sclerosus, and interstitial granulomatous dermatitis is still debated.
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PMID:[Dermatological aspects of Lyme borreliosis]. 1739 84

Liposomal pegylated doxorubicin is an encapsulation form of doxorubicin, with an improved pharmacokinetic profile and the ability to selectively accumulate into tumor tissue. As a result, the tolerated dose of the drug can be increased, followed by a reduced incidence of neutropenia and cardiotoxicity in comparison to doxorubucin treatment. However, a common adverse dose-schedule limiting effect of the treatment is palmoplantar erythrodysesthesia syndrome. In this retrospective study we included six patients hospitalised in the University Hospital of Zurich during the last 2 years, in connection with side effects caused by pegylated liposomal doxorubicin. These patients received this chemotherapeutic agent for treatment of various malignancies such as breast cancer, ovarian cancer, mycosis fungoides and cutaneous B-cell lymphoma. Three of six patients in this study developed classical palmoplantar erythrodysesthesia, one developed palmoplantar erythrodysesthesia associated with extensive bullous disease, one developed eruption of lymphocyte recovery syndrome and one developed intertrigo like dermatitis with stomatitis. Pegylated liposomal doxorubicin induces various skin reactions including palmoplantar erythrodysesthesia syndrome. However, the exact clinical presentation might depend on pre-existing skin diseases.
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PMID:Skin problems associated with pegylated liposomal doxorubicin-more than palmoplantar erythrodysesthesia syndrome. 1921 58

The known spectrum of skin manifestations in cutaneous Lyme disease is continuously expanding and can not be regarded as completed. Besides the classical manifestations of cutaneous borreliosis like erythema (chronicum) migrans, borrelial lymphocytoma and acrodermatitis chronica atrophicans evidence is growing that at least in part also other skin manifestations, especially morphea, lichen sclerosus and cases of cutaneous B-cell lymphoma are causally related to infections with Borrelia. Also granuloma annulare and interstitial granulomatous dermatitis might be partly caused by Borrelia burgdorferi or similar strains. There are also single reports of other skin manifestations to be associated with borrelial infections like cutaneous sarcoidosis, necrobiosis lipoidica and necrobiotic xanthogranuloma. In addition, as the modern chameleon of dermatology, cutaneous borreliosis, especially borrelial lymphocytoma, mimics other skin conditions, as has been shown for erythema annulare centrifugum or lymphocytic infiltration (Jessner Kanof) of the skin.
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PMID:The expanding spectrum of cutaneous borreliosis. 1935 23


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