Panniculitis-like lymphoma clinically manifesting as alopecia

A 45-year-old female patient discussed herein had a multifocal, alopecia areata of inflammatory character. Histological findings first suggested histiocyte cytophagic panniculitis, although a "burned-out" panniculitis-like T-cell lymphoma could not be excluded. After a two-year follow-up period assessment of the T-cell receptor gene rearrangement verified the diagnosis of subcutaneous panniculitis-like T-cell lymphoma. This case is interesting because of its isolated manifestation and a long-standing, benign course of the disease.     

儿童种痘水疱病样皮肤T淋巴细胞瘤1例及其文献复习

目的：通过归纳、分析种痘水疱病样皮肤T淋巴细胞瘤的免疫组化特点、治疗方案及预后,提高临床医生对本病的诊断治疗水平。方法：报道本科1例种痘水疱病样皮肤T淋巴细胞瘤病例,并应用万方数据库及PubMed数据库查找已发表且患者为中国人的文献,分析、归纳其免疫组化特点、治疗方案及预后。结果：种痘水疱病样皮肤T淋巴细胞瘤好发于儿童,多为女性儿童,亦可见于成人,本病免疫组化CD3、CD8、CD45、EBER、TIA-1、Ki-67阳性率高,CD4、CD30、CD56阳性率较低,免疫调节治疗疗效较好。结论：种痘水疱病样皮肤T淋巴细胞瘤多见于女性儿童,免疫组化CD3、CD8、CD45、EBER、TIA-1、Ki-67可作为诊断本病的指标之一。本病无特效疗法,但调节免疫治疗可考虑作为一线治疗方案。     

Non-responsive coeliac disease: what to do?

Coeliac disease (CD) is the most common cause of villous atrophy and is increasingly recognized. The majority of CD patients responds to a gluten-free diet (GFD). However, some patients experience persistence or recurrence of symptoms despite a GFD. These patients require further diagnostic workup. We describe a 62-year-old female with recurring symptoms attributed to refractory coeliac disease (RCD) type I. A 66-year-old patient with a similar history had aberrant intraepithelial lymphocytes characteristic for RCD type II in her duodenum. Furthermore, in a third CD patient described here, microscopic colitis was responsible for diarrhoea that persisted despite strict dietary adherence. Microscopic colitis is strongly associated with CD and should be considered in patients with this disease. On the basis of these three illustrative case studies, we discuss the causes of non-responsive CD and their respective diagnostic workup.     

原发于肺的NK/T细胞恶性淋巴瘤1例

目的：提高临床医师对NK/T细胞恶性淋巴瘤的认识。方法：通过病史采集、有创作、病理和免疫组化确诊该病。结果：原发于肺部的NK/T细胞恶性淋巴瘤比较罕见,恶性程度高,病程进展快,治疗效果及预后差。结论：对疑似病例进行有创检查,结合病理及免疫组化有助于明确诊断。     

Kikuchi's histiocytic necrotising lymphadenitis: a benign disorder--not to be confused with malignant lymphoma

A 26-year-old woman who later turned out to have the rarely seen histiocytic necrotising lymphadenitis of Kikuchi was twice diagnosed incorrectly with malignant T-cell lymphoma. She was treated with standard chemotherapy, whereas Kikuchi's disease has a self-limiting course. Fear for recurrent lymphoma greatly affected the patient's life until the proper diagnosis was ultimately made. This occurred after the patient herself had seen in her dossier that the diagnosis 'Kikuchi's histiocytic lymphadenitis' had been proposed by two pathologists of the consulted regional lymphoma board in the past, but had been rejected by the board after external consultation.     

Maladie cœliaque de l’adulte : actualités du régime sans gluten

Adult celiac disease (ACD) is an autoimmune enteropathy induced by the ingestion of gluten in genetically predisposed individuals. Its classical lesion is total or subtotal villous atrophy in at least the proximal small bowel, responding to the dietary exclusion of prolamins, the alcohol-soluble fractions of four cereals reputed to be toxic: gliadins of wheat gluten, secalins of rye, hordenins of barley, and - probably to a lesser extent - avenins of oats.Gluten-free diet (GFD) is based on the exclusion of any food containing one or another of these four toxic cereals, and their possible replacement by others such as rice and maize. That oats is well-tolerated by adult celiacs is presently strongly suggested, but its long-term (more than 5 years) harmlessness remains to be proved. GFD is a constraining diet for patients and - in terms of follow-up - for dieticians and physicians. Indeed, wheat gluten is present in a huge number of food-stuffs, products and culinary preparations (basic food as bread ans pastes, as well as cooked dishes, sweets, starchy food, icecreams, chocolate, some beers and appetizers. ACD patients have to know how to read product stickers to detect gluten even in trace amounts. Strict adherence to a GFD is difficult, particularly due to the subsequent decreased conviviality and high cost of replacement products. Prognosis of ACD under a strict and supervized GFD is excellent: the clinical response preceeds the biological and pathological ones. In adult celiacs clinically non-responding after a 3 month-diet period, poor adherence must be at first looked for. Primary or secondary true resistance to a GFD (refractory sprue: non-response to a 6-12 month strict and regularly supervized GFD) occurs in 5 to 8% of cases. Pseudo-refractory sprue is due to diet transgressions, more often unacknowledged than unvoluntary, but can result in true refractory sprue and its complications including lymphoma and death related or not to lymphoma. In adult celiacs, an at least 5 year-long strict GFD significantly decreases the risk of malignancy, especially T-cell lymphoma, and death. In the next future, the discovery of the toxic peptidic sequence of gliadin remains an interesting issue of potential importance for manufacturing strictly atoxic food-stuffs. Otherwise, the recent experimental observation that a bacterial prolyl endopeptidase causes degradation and loss of the antigenicity of a 33-aminoacid immunodominant peptide of α2-gliadin might ultimately find therapeutic relevance.     

Tropical or non-tropical sprue?

A 57-year-old Dutch man presented with weight loss and fatigue 6 months after a visit to West Papua, when he had suffered from serious diarrhoea. Macrocytic anaemia and vitamin B12 deficiency were diagnosed. A gastroduodenoscopy with biopsies of the small intestine was performed revealing no macroscopic abnormalities but partial villous atrophy was found microscopically, suggesting tropical sprue or coeliac disease. Antibodies against endomysium and tissue transglutaminase were negative, ruling out coeliac disease. The patient was successfully treated with vitamin B12, folic acid and doxycycline. This case shows that tropical sprue should be considered in the differential diagnosis of chronic diarrhoea in patients with a history of travel in tropical regions. The most frequent medical problem that travelers to the tropics experience is diarrhoea with an incidence of 30%. A small proportion of these patients eventually present with chronic diarrhoea. At that moment, the relation to their previous travelling may not be immediately clear. One of the causes of this chronic diarrhoea to be considered is tropical sprue.     

难治性高血压的病因及治疗进展

难治性高血压属于高血压疾病中的一种特殊类型,严重危害患者的身体健康,所以,有必要针对此疾病展开深入的研究与分析。难治性高血压疾病的病因较多,其临床治疗关键要积极转变患者的既有生活方式,使患者接受科学的诊断与针对性的治疗措施,结合患者病情,积极开展介入治疗亦或是药物联合治疗,进而改善患者的临床症状,控制病情发展,提高预后效果。基于此,本文将难治性高血压作为主要研究内容,重点阐述其病因与治疗进展,致力于进一步提高其临床治疗效果,为广大临床工作者提供一定的参考依据。     

Primary T-cell lymphoma of the pancreas in children

Involvement of the gastrointestinal tract is frequently reported among the extranodal sites of non-Hodgkin's lymphoma, but primary lymphoma of the pancreas is very rare. We report the case of a 11-year-old boy explored for epigastric pain with fever leading to the diagnosis of high-grade primary non Hodgkin's T-cell lymphoma, originating from pancreas. This pancreatic lesion is documented by sonography, CT and MRI. This patient died quickly with sever infection.     

Intestinal T cell lymphoma in coeliac disease. A case report

Primary intestinal T-cell lymphomas account for about 5% of all primary gastrointestinal lymphomas and are mostly associated with coeliac disease. Intestinal lymphomas are usually discovered during the 4th to 5th decade and constitute an exceptional complication of coeliac disease. This lymphoma is generally a high grade pleomorphic cell lymphoma with large cells. Our case concerned a 30 year old woman with a history of coeliac disease who developed a gastro-enteromesenteric lymphoma. Anatomopathological and immunohistochemical analysis showed an enteropathy associated T-cell lymphoma. We suggest that patients with coeliac disease unresponsive to a gluten free diet or with deteriorating clinical condition be investigated for complications such as enteropathy associated T-cell lymphoma. It is also important to avoid this complication by detecting asymptomatic form of coeliac disease that needs a gluten-free diet.     

Hypercalcemia due to adult T-cell lymphoma in a man from Surinam

A 48-year-old male patient from Surinam presented with anorexia, nausea and weight loss. An extreme hypercalcaemia of 5.08 mmol/l was found. Further diagnostic investigations showed that this patient had a HTLV-1 positive adult T-cell leukaemia/lymphoma (ATL/L). This is often associated with multilobularly nucleated lymphocytes, bone destruction and hypercalcaemia. Skin localisations are frequently observed. The combination of cytomorphology, immunophenotyping, HTLV-1 seropositivity and clinical findings points to the diagnosis. The patient was treated with 6 courses of chemotherapy consisting of cyclophosphamide, doxorubicin, teniposide, prednisone, vincristine and bleomycin. Upon inquiry it appeared that he had died elsewhere. Chemotherapy induces a short-lived remission in a minority of ATL/L patients. Antiviral therapy (alpha interferon, zidovudine) might offer new possibilities.     

Peritoneal catheter site metastasis in a patient with advanced ovarian cancer

Refractory ascites can significantly reduce quality of life in patients with advanced stage ovarian cancer. Paracentesis is a commonly used procedure for the management of refractory ascites. However, there are potential risks associated with paracentesis such as pain, infection, and injury to intra-abdominal organs. In an effort to decrease the number of serial paracentesis, placement of intraperitoneal catheters has been described for the management of intractable ascites. Here we describe a case of metastasis involving a palliative peritoneal catheter in a patient with recurrent ovarian cancer.     

Primary CNS lymphoma presenting as multiple sclerosis. A case study

A case is described of a patient who presented with multifocal neurologic disease compatible with a diagnosis of multiple sclerosis. The predominance of cognitive abnormality was unusual in this case. It was not until the patient's rapid clinical deterioration and radiographic findings mandated surgical intervention that a disseminated tumor involving the leptomeninges was identified and a diagnosis of primary CNS lymphoma was made. Postmortem examination confirmed the diagnosis of primary CNS lymphoma, and the classical pathologic findings of multiple sclerosis were also seen. This report illustrates interesting parallels in the clinical differentiation of these two diseases.     

T gamma-lymphoproliferative disease

The case of a rare type of T-cell malignant lymphomas, clinically with a relatively favorable course, a T gamma-lymphoproliferative disease (T gamma-cell chronic lymphocytic leukaemia) was presented. The cytomorphological, cytochemical, immuncytochemical and cytogenetical markers and functional tests of the peripheral blood lymphocytes from the patient were tested. The leukaemic cells with light- and electron microscopy showed the so called LGL (large granular lymphocyte) morphology with multifocal reactions of acid hydrolase enzymes. These cells also expressed IgG-Fc-receptor, CD 8 monoclonal antibody positivity and a monoclonally rearranged T-cell receptor gen expression. Functionally the patient's lymphocytes developed a blastic response to the T-cell mitogen Concanavalin A (ConA), they suppressed the immunoglobulin production of B-lymphocytes in co-cultures and had a normal NK-activity but decreased ADCC values. The patient was diagnosed by blood, bone marrow and lymph node examination and does not need any therapy. This case was published because of it's diagnostic, immunological and prognostical interests.     

Experience with fludarabine treatment and review of the literature

INTRODUCTION: Fludarabine is the most commonly used purine analog, its mechanism of action is complex. Fludarabine inhibits DNA synthesis, acts on non-dividing (G0 phase) cells influencing apoptosis. PATIENTS, RESULTS, CONCLUSIONS: In our institute 47 patients were treated with fludarabine or fludarabine based combination chemotherapy. Fludarabine was given in 19 patients with chronic lymphocytic leukaemia (CLL), complete remission (CR) was achieved in one case, partial remission (PR) was obtained in 10 patients. Fludarabine was more effective in patients who received less intensive chemotherapy prior to fludarabine therapy and in those patients who had less advanced diseases. Elderly patients (over sixty years of age) also responded to fludarabine therapy. Fludarabine and cyclophosphamide combination (FCy) were used in three lymphocytic lymphoma patients, two of them obtained PR, in the third case the disease progressed. Fludarabine + mitoxantrone (Novantrone) + dexamethasone (FND) regimen was administered in nine patients who were previously heavily treated (one patient with B-CLL, one with T-CLL, one with peripheral T-cell lymphoma and six with indolent B-cell lymphoma). More patients and longer follow up is needed to determine the efficacy of FCy and FND protocol. FLAG-IDA (fludarabine, high dose Ara-C, granulocyte colony-stimulating factor, idarubicin) was applied in 16 acute leukaemia patients with poor prognosis including therapy refractory and relapsing cases. Three CR and two PR, one CR and three PR was achieved in nine patients with acute myeloid leukaemia and in seven patients with acute lymphoid leukaemia, respectively. For this reason, despite the short period of remission, this regimen can be recommended to patients who are candidate for stem cell transplantation.     

Viruses and cancer. Causal associations.

This review first considered some general problems in establishing causal links between a virus and a human cancer and offered some guidelines in the pursuit of this objective. Second, it reviewed the current causal associations for several candidate oncogenic viruses in relation to the tumors with which they are associated. These include Epstein-Barr virus in relation to Burkitt's lymphoma, nasopharyngeal carcinoma, Hodgkin's disease, and non-Hodgkin's lymphoma; hepatitis B and C viruses in relation to hepatocellular carcinoma; human T-cell leukemia/lymphoma virus type 1 and atypical leukemia/lymphoma; and human papilloma viruses in relation to cervical carcinoma. For some, the causal relationship is strong: hepatitis B virus with hepatocellular carcinoma, and human T-cell leukemia/lymphoma virus with adult T-cell leukemia/lymphoma. For one, the causal relationship is moderate: Epstein-Barr virus with African Burkitt's lymphoma. For others it is incomplete or inconclusive: Epstein-Barr virus with Hodgkin's disease and non-Hodgkin's lymphoma, and hepatitis C virus with hepatocellular carcinoma. Current techniques do not permit an answer for some: human papilloma virus with cervical carcinoma.     

Clinical reasoning and decision making in practice. A 41-year old with periodic fever of unknown origin

A 41-year-old man presented with unexplained bleeding from the right tonsil. He subsequently developed periodic fever, cervical lymphadenopathy and hepatosplenomegaly. Despite extensive bacteriological, serological and radiographic investigations for infectious disease, rheumatic disease and malignancy no diagnosis was made. Although the fever pattern was very suggestive of Pel-Ebstein fever--commonly associated with lymphoproliferative disease--multiple biopsies of lymph nodes, bone marrow, tonsils and liver all proved negative. Empirical glucocorticoid therapy gave some temporary improvement lasting for a month. Splenectomy or splenic biopsy was not carried out because of the risk of excessive bleeding. Eventually the patient died of multi-organ failure and sepsis. At autopsy, a T-cell lymphoma with an unusual phenotype and focal involvement of bone marrow, liver and spleen was found. Clinicians are sometimes faced with the dilemma of whether to perform multiple, invasive and possibly harmful diagnostic tests or to start empirical therapy. Empirical therapy may only be started if the diagnosis has been made on strong clinical grounds and, if this is not the case, only after further diagnostic tests. The question of whether a potentially harmful diagnostic test is justified depends on the clinical course, the sensitivity and specificity of the test and the therapeutic possibilities.     

Current concepts on celiac disease physiopathology

Celiac sprue (CS) is defined as a chronic small bowel malabsorption disorder caused by ingestion of gluten, affecting those genetically predisposed individuals. It is characterized by intestinal villi atrophy, increased number of intraepithelial lymphocytes and extense inflammatory infiltrate in the intestinal lamina propria. The role of gluten as responsible for the intestinal damage seen in CS patients is clear, however, the physiopathological mechanisms involved are still unknown. Several factors and theories have been proposed: 1) Genetic predisposition, based on the association to mendelian factors as well to the presence of particular major histocompatibility complex (MHC) haplotypes in CS patients; 2) Immunological factors, that consider the derangements that occur in the immune response of CS patients, and 3) Gliadin partial deamination by the tissular transglutaminase (tTG). In an effort to explain all these complex mechanisms, recently, all these theories have been unified, yielding one complex physiopathogenic mechanism that we tray to explain in the present review.     

What kind of clinical, epidemiological, and biological data is essential for the diagnosis of Lyme borreliosis? Dermatological and ophtalmological courses of Lyme borreliosis

Lyme borreliosis (BL) is a multisystem infectious tick-transmitted disease. The diversity of Borrelia burgdorferi is the reason for a wide spectrum of dermatological and ophthalmologic presentations between patients from Europe and from other countries. In Europe, the main manifestations are dermatological. During the early stage, the diagnosis is clinical: finding erythema migrans (EM) a few days after a tick bite is sufficient; several EM mean an early-disseminated disease. Borrelial lymphocytoma (only in Europe) is a solitary nodule or plaque (earlobe, nipple, scrotum), which appears during the second stage. The diagnosis relies on clinical and histological findings (B-cell infiltration) and a positive serological test. It is sometimes difficult to make the difference between BL and B-cell lymphoma and pseudo lymphoma; an empirical antibiotic trial period will be helpful for the diagnosis in this case. During the late stage, the clinical evolution of acrodermatitis chronica atrophicans is progressive: inflammatory then atrophic lesions appear, often on the hands, limbs, or feet. The diagnosis is made on histological findings (T-cell infiltration) and a positive serological test. The relationship between BL and morphea or lichen sclerosus was not demonstrated according to the latest reports. Ocular manifestations are rare events occurring during every stage of the disease. A wide spectrum of presentations is possible (uveitis and optic neuritis). BL is responsible for ocular infection or inflammation. A neurological presentation is often associated with the ocular manifestation. Proving the diagnosis is often difficult because of these polymorphous manifestations.     

Collagen sprue--a rare form of adult celiac disease]

The authors report the first case of collagenous sprue in Hungary. This condition is characterized by coeliac type small bowel malabsorption, resistant to gluten free diet and other therapeutic efforts, associated with poor prognosis. The diagnosis depends on the histological demonstration of extensive collagenization of the lamina propria in the flat jejunal mucosa. This disease must be kept in mind at differential diagnosis of chronic diarrhoea with progressive malabsorption, especially if it is resistant to gluten withdrawal than conventional coeliac disease.