Primary cutaneous T-cell lymphoma presenting as mycosis fungoides with a T-/null-cell phenotype: report of two cases.

Variations in the clinical and histological presentation of cutaneous T-cell lymphoma (CTCL) can hamper diagnosis. We report two cases of a novel presentation of CTCL characterized by an aberrant immunophenotype with complete loss of pan T-cell antigens including T-cell receptor ß chain and showing the clinical and histopathological appearance of erythrodermic and plaque-stage mycosis fungoides.

Serum value of procalcitonin as a marker of intestinal damages: type, extension, and prognosis.

BACKGROUND: Ischemic and necrotic damages are complications of digestive diseases and require emergency management. Nevertheless, the decision to surgically manage could be delayed because of no sufficiently preoperative accurate marker of ischemia diagnosis, extension, and prognosis. METHODS: The aim of this study was to assess the predictive value of serum procalcitonin (PCT) levels for diagnosing intestinal necrotic damages, their extension, and their prognosis in patients with ischemic disease including ischemic colitis and mesenteric infarction by a gray zone approach. Between January 2007 to June 2014, 128 patients with ischemic colitis and mesenteric infarction (codes K55.0 and K51.9) were operated, for whom data on PCT were available. We perform a retrospective, multicenter review of their medical records. Patients were divided into subgroups: ischemia (ID group) versus necrosis (ND group); the extension [focal (FD) vs. extended (ED)] and the vital status [deceased (D) vs. alive (A)]. RESULTS: PCT levels were higher in the ND (n = 94; p = 0.009); ED (n = 100; p = 0.02); and D (n = 70; p = 0.0003) groups. With a gray zone approach, the predictive thresholds were (i) for necrosis 2.473 ng/mL, (ii) for extension 3.884 ng/mL, and (iii) for mortality 7.87 ng/mL. CONCLUSION: In our population, PCT could be used as a marker of necrosis; especially in case of extended damages and reflects the patient's prognosis.

Nonuraemic calciphylaxis: response to treatment with pamidronate and negative pressure therapy.

Calciphylaxis is a rare cause of skin ulcerations and necrosis in patients with both normal renal and parathyroid function. Although calciphylaxis appears to be on the increase, treatments are mainly empirical, especially for wound care. The lesions in calciphylaxis are typically very painful and carry a high risk of infection, with sepsis being the leading cause of death in this serious disease. We report two cases of nonuraemic calciphylaxis, which responded to treatment with pamidronate and wound management by negative pressure system.

Innate mucosal-associated invariant T (MAIT) cells are activated in inflammatory bowel diseases.

Inflammatory bowel diseases are characterized by a deregulated immune response targeting the gut bacterial flora. Mucosal-associated invariant T (MAIT) cells are major histocompatibility complex (MHC) class Ib-restricted innate-like lymphocytes with anti-bacterial functions. They display an effector/memory phenotype and are found in large numbers in the blood, mucosae and liver. They have also been implicated in inflammatory diseases such as multiple sclerosis. Therefore, we aimed to analyse the possible involvement of MAIT cells in Crohn's disease (CD) and ulcerative colitis (UC). To this end, a phenotypical and functional analysis of MAIT cells isolated from the blood of healthy subjects, CD and UC patients was undertaken. MAIT cells were also quantified in ileal biopsies of CD patients. The frequency of blood MAIT cells was specifically reduced in IBD patients compared with healthy donors, whereas it was dramatically greater in the inflamed versus healthy tissue. MAIT cells were activated as they expressed significantly more the Ki67 antigen, and this was accompanied by phenotypical changes such as increased expression of natural killer (NK)G2D and B and T lymphocyte attenuator (BTLA). Finally, in-vitro-activated MAIT cells from CD and UC patients secreted significantly more interleukin (IL)-17, together with a decreased interferon (IFN)-γ in CD but an increased IL-22 in UC. These data show that MAIT cells are activated in IBD, which results in an increased recruitment towards the inflamed tissues, an altered phenotype and a switch in the pattern of cytokine secretion. This is the first demonstration that MAIT cells are immune players in IBD, whose precise functions in this context need to be addressed.

Sudden death caused by aortic dissection in a patient with polycystic kidney disease.

A 43-year-old man presented at the emergency medical unit with chest pain. The results of a clinical examination were normal, apart from sternum pain (without radiation) on palpation. The patient had no respiratory problems and the pain was relieved by paracetamol. The electrocardiogram, laboratory tests and chest X-ray were normal. However, the man was found dead the next morning. In the autopsy, we noted the presence of haemopericardium, aortic dissection (starting from the vessel's origin and extended to the aortic arch and on through the diaphragm), polycystic kidney disease and liver cysts. In adult autosomal dominant polycystic kidney disease (ADPKD) patients, the main causes of death are ruptured intracerebral aneurysms, coronary artery disease, congestive heart failure, valvular heart disease and ruptured abdominal aortic aneurysms. Aortic dissection is considered to be rare cause of sudden death in ADPKD sufferers. ADPKD can have serious consequences for the vascular system. The families of confirmed ADPKD sufferers must be informed and screened as early as possible, in order to prevent renal and cardiovascular complications.

Letter: A case of generalized guttate psoriasis induced by etanercept with relapse after abatacept.

Abatacept is the first in a new class of agents for the treatment of rheumatoid arthritis. We report a case of guttate psoriasis in a patient treated with abatacept for rheumatoid arthritis. This patient had the same reaction with etanercept in the past.

Gastric metastases. An endoscopic series of ten cases.

We report a series of ten cases of the clinical, endoscopic and pathological features of gastric metastases. Patients were six women and four men between 54 and 88 years old, with gastric metastases from breast carcinoma (4), lung carcinoma (4) and melanoma (2). Patients underwent an upper gastrointestinal endoscopy for epigastralgia (2), hematemesis (2), dysphagia (1) and anemia (5). On endoscopy, tumors appeared as nodules with a central ulceration (5), an ulceration (4) or simulating linitis plastica (1). Metastases were located in the cardia (2), fundus (5) and antrum (3). Primary tumors had been diagnosed between one day and 20 years before upper endoscopy. Eight patients had multivisceral metastases. The microscopic features of the gastric metastases resembled a primary gastric cancer in eight cases. Thanks to clinical data, the pathologist confirmed the diagnosis of gastric metastases on immunohistochemistry. Nine patients died in the eight-month follow-up period. Gastric metastases are rare, occur at a late stage of the neoplastic disease, and have a poor prognosis. Diagnosis of gastric metastases is difficult because they simulate primary gastric cancer on endoscopy and on microscopic examination. A correct diagnosis is based on good communication between gastroenterologists and pathologists.

[A case of pancreatic schwannoma successfully treated by enucleation]. / Schwannome pancréatique traité avec succès par énucléation : à propos d'un cas.

Benign pancreatic schwannoma is a very rare cystic and solid tumor, which poses a diagnostic and therapeutic challenge. Herein, we present a case of 4-cm solitary benign schwannoma of pancreatic uncinate process, with pancreas divisum, incidentally discovered in a 53-year-old woman. Preoperative diagnosis was established by endoscopic ultrasound and fine needle aspiration. Simple enucleation of the tumor from the surrounding parenchyma was successfully performed. No complications were found after the operation.

[Eosinophilic dermatosis associated with hematological disorders: A clinical, histopathological and immunohistochemical study of six observations]. / Dermatose éosinophilique associée aux hémopathies : étude clinique, histopathologique et immunohistochimique de six cas.

BACKGROUND: Eosinophilic dermatosis of hematologic disease (EDH) or insect bite-like reaction is a pruritic dermatitis described mostly in patients with chronic lymphocytic leukaemia (CLL). We describe six patients with the disorder in association with CLL and other blood dyscrasias. PATIENTS AND METHODS: We reviewed the medical records of patients with EDH seen between 2004 and 2009 in our department and re-examined histological slides. RESULTS: Mean age at dermatosis onset was 75.6 years and the sex ratio was 1. There were three CLL, two mantle-cell lymphomas and one MALT-type lymphoma. The dermatitis was quite polymorphic, with erythematous papules, wheals and plaques. The initial skin lesions appeared at the same time as or after the diagnosis of haematological neoplasm. Their reappearance heralded relapse of the blood disease in three cases. Histologically, all lesions had a dense dermal infiltrate of small, mostly CD4+ T-cells, with numerous eosinophils. In three patients, there was marked folliculotropism, resembling folliculotropic T-cell lymphoma. In most cases, EDH disappeared after appropriate chemotherapy for the blood disorder. DISCUSSION: Our cases show that the clinical expression of EDH is quite polymorphic. Its appearance may precede relapse of or may indicate prompt search screening for blood dyscrasia. The most efficient treatment of this dermatosis appears to be specific chemotherapy for the blood dyscrasia. There is reason to believe that a population of T-helper 2 (Th2) lymphocytes, reactive to malignant B-cells, induces tissue eosinophilia, mainly through production of interleukin (IL)-5, among other cytokines. Eosinophils appear to be the main effector cells.

Endoscopic or surgical ampullectomy for intramucosal ampullary tumor: the patient populations are not the same.

PURPOSE: To evaluate and compare the results of endoscopic ampullectomy (EA) and surgical ampullectomy (SA) for benign tumors. MATERIAL AND METHOD: From 2005 to 2018, 66 patients were eligible for ampullectomy (adenoma, ultrasound grading≤T1, N0). The principal endpoint was the rate of complete resection: R0 resection without local recurrence and no recurrence after a single operative procedure and with final pathology confirming the operative indications. Secondary endpoints were: morbidity, mean length of stay (LOS), preoperative and final pathology results, complete (or incomplete) resection, mean duration of follow-up, local recurrence rate and late complications (biliary stricture). RESULTS: Among the 41 patients eligible for EA, 36 ampullectomies were performed, while five were referred for SA because of intracanalar invasion that had not been diagnosed initially. The rate of complete treatment was 48% (13/27), the morbidity was 10%; the LOS was 3.3days; no adenomatous lesion was found on the definitive pathology in 9 patients (25%). The resection was R1 in 30% but there were no R2 resections. The median duration of follow-up was 20months, the rate of local recurrence was 22% and the rate of late biliary stricture was 2%. Among the 30 patients who were eligible for SA, (25 plus the five referred by endoscopists after intracanalar invasion was found), SA was eventually performed in 24: five patients were converted to pancreatoduodenectomy, one patient was found to have metastatic disease and had no resection. An extemporaneous frozen-section pathology examination was performed in 22 of the 24 SA patients and confirmed healthy margins on the bile ducts and pancreatic ducts. The rate of complete treatment was 71% (17/24), morbidity was 35%; LOS was 10days; seven patients (29%) were found to have invasive adenocarcinoma, and six of these (86%) underwent subsequent pancreatoduodenectomy. None of the resections were R1 or R2; median follow-up was 21months; there was no local recurrence; biliary stricture rate was 8%. CONCLUSION: The short-term and long-term results of patients undergoing endoscopic or surgical ampullectomy are different. The lesions resected by EA were less advanced, with simpler postoperative course. The lesions for which SA was performed were more advanced, and had more morbid sequelae, but with a better rate of complete treatment and better long-term results.

Pathological features of temporal arteries in patients with giant cell arteritis presenting with permanent visual loss.

BACKGROUND: Permanent visual loss (PVL) is the most feared complication of giant cell arteritis (GCA), and its risk factors are still unclear. OBJECTIVES: The aim of our study was to assess the pathological features predictive of PVL on temporal artery biopsy (TAB) specimens in patients with GCA. METHODS: The slides of 391 TAB specimens from patients with GCA were reviewed by two pathologists without clinical information. RESULTS: A total of 29 patients (26 females and 3 males, mean age 78.3 years) presented with unilateral PVL at the onset of the disease, and 362 patients (258 females, 104 males, mean age 74.7 years), did not. The pathological features strongly predictive for PVL were the presence (p = 0.003), number (p = 0.001) and aggregates of giant cells (p = 0.001), presence of plasmocytes (p = 0.002), thickened intima (p = 0.007), neoangiogenesis (p = 0.001) and degree of arterial occlusion (p = 0.006). Presence of neutrophils, eosinophils, parietal necrosis, calcification in the arterial wall and disruption of the internal elastic membrane were similar in both groups. Total obstruction of the arterial lumen by a thrombus, intensity of the inflammatory cells infiltration and inflammation of small vessels, nerves and veins surrounding the temporal artery were not associated with blindness. In multivariate analysis, only giant cells remained significantly associated with PVL. CONCLUSION: Giant cells are strongly associated with PVL, with a significant gradient between great risk and large number of giant cells. However, PVL was neither associated with the intensity of the inflammatory infiltrate, nor with the presence of arterial thrombosis.

Procalcitonin at the onset of giant cell arteritis and polymyalgia rheumatica: the GRACG prospective study.

OBJECTIVES: An epidemic pattern has been reported for GCA and PMR. Immunological studies have shown that an unknown antigen activates the dendritic cells of the adventitia and the type 4 toll-like receptors. Procalcitonin (PCT) is an early marker of bacterial infection. The goal of the study was to assess the level of PCT in GCA and PMR at the onset of the disease. METHODS: Patients diagnosed during the 2002-06 period were randomly selected. All the 46 patients fulfilled the ACR or the Hunder criteria, and all blood samples were taken before steroid therapy. RESULTS: PCT was normal in all patients. PCT was slightly increased in men (0.087 +/- 0.023 microg/l) compared with women (0.066 +/- 0.027 microg/l) (P = 0.009), and in PMR (0.092 +/- 0.027 microg/l) compared with GCA (0.068 +/- 0.026 microg/l) (P = 0.018). There was no significant correlation with inflammation markers. CONCLUSIONS: These results are not in favour of a bacterial trigger for GCA or PMR. Increased PCT levels in patients with inflammatory syndrome, GCA-PMR symptoms and negative temporal artery biopsy may rule out the diagnosis of GCA and PMR.

[Dead children born: Current regulation, inscription to registry office differs]. / Décès périnatal : réglementation actuelle, inscription à l'état civil et devenir du corps.

From a juridical point of view, in France, we become a "person" only when we are born "living" and "viable". These two criteria are necessary, but the threshold of viability is not defined by the law. The general education of registry office leaned on a circular fixing a << threshold of viability >> itself based on a recommendation of the Worldwide Organization of Health (WHO). The fetus was considered as viable after a term of twenty-two weeks of amenorrhea or if it had a weight over or equal to 500 grammes. The inscription to Registry office differs, as well as the taking care of the body of the child, depending on whether he was born living, viable and living and not viable, dead and viable, or dead and not viable. In France, the civil officer established an act of child declared lifeless when the child was born living but not viable or when the child is death - born but viable. However, parents of not viable and born dead children, often close to the threshold of viability, also liked to acquire an act of lifeless child, to be able to organize funeral has child lifeless and to inscribe it in their family record book. The act of child declared lifeless allows to inscribe the child on the family record book if the parents wish and give to the families the delay of ten days to claim the body. By judgment of February 6th, 2008, the Supreme Court of appeal cancelled rulings where the threshold had been kept to refuse the deliverance of an act of lifeless child. Her Supreme court of appeal considers that law does not impose de threshold from which the recognition of the status of lifeless child would be possible. Since the decrees of August, 2008, there is no border anymore of minimum of term or weight. Consequently, the lifeless born children after an unprompted delivery or a medical break, the pregnancy can be inscribed on the civil record. On the other hand, it is not possible for the precocious wrong coat and the termination of pregnancy.

[Hepatic Epithelioid hemangioendothelioma: a rare liver tumor]. / L'hémangioendothéliome épithélioïde du foie: une tumeur hépatique rare.

Epithelioid hemangioendothelioma of the liver is a rare primary malignant tumor of vascular origin. It most frequently occurs in middle age adults and clinical findings are non-specific. Histological diagnosis may be difficult to achieve with lesions containing a large myxoid component, and immunohistochemical staining is required. A few imaging features may suggest the correct diagnosis, and radiologists should be familiar with these findings. We will illustrate the potential role of PET-CT at the time of initial presentation. Prognosis is variable and optimal management is based on a case by case analysis, ranging from clinical and imaging follow-up to liver transplantation.

[Low-molecular-weight heparin-induced bullous haemorrhagic dermatosis associated with cell-mediated hypersensitivity]. / Dermatose bulleuse hémorragique associée à une réaction d'hypersensibilité retardée sous héparine de bas poids moléculaire.

BACKGROUND: Heparin-induced bullous hemorrhagic dermatosis is a rare, recently described side-effect of subcutaneous heparin injection. We describe a patient simultaneously presenting distant haemorrhagic bullae and eczematous reaction at the low molecular-weight heparin (LMWH) injection sites. PATIENTS AND METHODS: Subcutaneous enoxaparin sodium was initiated in a 51-year-old patient and was replaced a few days later by tinzaparin sodium. Forty-eight hours later, annular, erythematous and vesicular plaques appeared at the injection sites (thighs). Small hemorrhagic bullae were noted on the abdominal skin at the same time. Skin biopsies revealed respectively eczematous dermatitis and an intraepidermal blister filled with red blood cells. Direct immunofluorescence was negative. Standard laboratory investigations and coagulation studies were unremarkable. Skin lesions disappeared ten days after discontinuation of LMWH. Patch tests and intradermal tests were negative. DISCUSSION: The case described herein shares the stereotypical clinical picture previously reported, namely small, multiple, haemorrhagic bullae on normal skin, appearing at remote sites five to 21 days after the start of subcutaneous heparin treatment. Despite the absence of clear management guidelines, it is obviously tempting to stop the heparin if there are too many bullae for fear of more clinically significant and dangerous mucous membrane lesions. The underlying physiopathological mechanism is poorly understood; no coagulation abnormalities were recorded. In addition, our patient presented an eczematous reaction at the injection sites, raising the possibility of a type IV hypersensitivity reaction. The association of these two cutaneous side effects of heparin is perhaps not purely coincidental.

[Bortezomib-induced eruption: Sweet syndrome? Two case reports]. / Toxidermie au bortézomib : syndrome de Sweet? Deux cas.

BACKGROUND: Bortezomib (Velcade) is a proteasome inhibitor used in the treatment of myeloma and other blood dyscrasias. We report the cases of two patients who developed a peculiar toxic rash suggestive of Sweet's syndrome while receiving bortezomib; one patient also presented giant mucous membrane ulcerations. PATIENTS AND METHODS: Case 1: bortezomib treatment was started in a 62-year-old man for mantle cell lymphoma. Ten days after the first treatment cycle, giant, painful oral ulcerations were noted but they resolved spontaneously. One week after the second cycle, further oral ulceration appeared, this time with a papulonodular skin rash. Histology showed neutrophilic dermal infiltrates in the skin with predominantly lymphocytic inflammation of the oral mucosa. Bortezomib was stopped and all lesions resolved with colchicine treatment. Case 2: a 46-year-old woman was receiving bortezomib treatment for plasma cell leukemia. A febrile skin rash appeared two days after the first treatment cycle but resolved spontaneously. After the first bortezomib injection during the next cycle, painful papules and nodules appeared on the trunk. The skin biopsy results were consistent with Sweet's syndrome. The lesions disappeared spontaneously. Dexamethasone was administered concomitantly with bortezomib in the ensuing cycles and there was no relapse of the skin lesions. DISCUSSION: Bortezomib-induced skin lesions are common and usually do not justify treatment withdrawal. Published observations of bortezomib-induced eruption occasionally show clinical and histological features of Sweet's syndrome, but there has been no mention of oral mucosal ulcerations. In our cases, these could be related to bortezomib-induced neutrophilic dermatosis.

[Surgical management of resectable hilar cholangiocarcinoma]. / Prise en charge chirurgicale du cholangiocarcinome hilaire résécable.

Hilar cholangiocarcinoma is a cancer with a poor long-term survival rate (20% at five years), even after curative resection (R0: surgical margins free of tumour). Recent advances have been made in the preoperative management of these patients: clinical imaging, such as magnetic resonance cholangiography, biliary drainage according to the type of hilar obstruction or laparoscopic assessment before portal vein embolization. Extended hemihepatectomy has recently been recognized as the standard curative treatment for hilar bile-duct cancer with concomitant resection of the portal-vein bifurcation as a radical resective procedure. Neoadjuvant-photodynamic therapy could be a new approach to treating hilar cholangiocarcinoma decreasing tumour margins, which is a major prognostic factor.

[Inflammatory fibroid polyp, a rare tumor of the appendix]. / Le polype fibroïde inflammatoire, une tumeur rare de l'appendice.

We report the rare occurrence of an inflammatory fibroid polyp of the appendix. The lesion was diagnosed in a 33-year-old woman presenting with abdominal pain, fever and localized tenderness in right iliac fossa on abdominal palpation. CT-scan showed an 8 cm appendiceal mass and a laparoscopic appendectomy was consequently performed. On microscopic examination, the tumor consisted of spindle cells dispersed in a loose fibromyxoid stroma containing numerous blood cells and inflammatory cells with abundant eosinophils. On immunohistochemistry, the spindle tumor cells were positive for vimentin, fascin and focally for CD34 and CD35. They were negative for smooth muscle actin, desmin, CD21, CD23, CD117 and S100 protein. Inflammatory fibroid polyp is a rare benign mesenchymal tumor of the gastrointestinal tract rarely reported in the appendix. This tumor shares some common pathologic features with the myofibroblatic inflammatory tumor but they are two different entities. The pathogenesis of this tumor remains unclear but fascin and CD35 immunoreactivity of the tumor cells suggests a probable dendritic cell origin.

[Gastric calcifying fibrous pseudotumor, a rare mesenchymal tumor of the stomach]. / La pseudotumeur fibreuse calcifiante gastrique, une tumeur rare de l'estomac.

We report a case of calcifying fibrous pseudotumor fortuitously discovered on abdominal CT-scan in a 50 year-old woman presenting with metrorragia. CT-scan showed a 2 cm hypodense nodular tumor of the gastric wall with granular calcifications. Upper endoscopy showed a nodular tumor along the lesser curvature of the gastric body, covered by normal gastric mucosa and biopsies were negative. A diagnosis of gastric stromal tumor was suspected and a partial gastrectomy was performed. On gross examination surgical specimen showed a firm, whitish nodular tumor measuring 2 cm in diameter. On microscopic examination the tumor was composed of whorls of dense hyalinized collagen bundles with a few fibroblasts. There were psammomatous calcifications and nodular aggregates of mononuclear inflammatory cells. Immunohistochemically, spindle cells stained for vimentin. They did not stain for smooth muscle actin, desmin, CD34, CD117, S100 protein, Ki-67 and ALK-1. Surgical resection of the tumor was complete. Patient has no evidence of disease with a follow-up of 12 months. Only seven cases of gastric calcifiying fibrous pseudotumors have been reported in the literature. These benign tumors are usually incidentally discovered. They have characteristic imaging and microscopic features and appear as a distinct clinicopathologic entity different from stromal tumors.