[Multicentric reticulohistiocytosis is a rare form of paraneoplasia].

A 59-year-old woman developed a rash and severe arthralgia, which primarily affected her fingers. She displayed digital arthritis and nodules on the hands, chest, face, and oral cavity. Blood samples were normal. Skin biopsies revealed histiocytic proliferation. The surface marker profile and clinical findings were consistent with multicentric reticulohistiocytosis, which may occur as a paraneoplastic phenomenon. On workup, she was diagnosed with an otherwise asymptomatic stage IVC fallopian tube cancer. She experienced little effect of prednisolone, but her condition improved on antineoplastic treatment.

Reactive granular histiocytosis secondary to arthroplasty prosthesis: a novel reaction pattern.

A reactive histiocytic infiltrate can be seen as an incidental finding in a lymph node biopsy from a patient with a history of joint arthroplasty. We report the case of a 74-year-old female who underwent surgical revision of a polyethylene-based right total knee prosthesis due to chronic wear. At the time of surgery, a soft tissue mass adjacent to the tibial prosthetic insert was noted and excised. Histopathologic examination revealed a sheet-like proliferation of large, histiocytoid cells within the subcutis and superficial fascia. The cells showed abundant eosinophilic, granular cytoplasm and small round bland nuclei. Immunohistochemical evaluation revealed the cells to be positive only for CD68. In addition, abundant PAS-positive cytoplasmic granules were found, and minute particles of polarizable material were noted intracellularly and scattered throughout the interstitium of the infiltrate. These findings were interpreted as consistent with a reactive, non-Langerhans cell histiocytosis secondary to the patient's polyethylene knee prosthesis. This finding appears to be a local correlate of the process previously described in regional lymph nodes as reactive granular histiocytosis. Dermatopathologists should be cognizant of this uncommon reaction pattern to avoid mistaking it for a neoplastic process.

[Paraneoplastic multicentric reticulohistiocytosis]. / Histiocytose multicentrique paranéoplasique.

BACKGROUND: Multicentric histiocytosis (MH) is a rare form of histiocytosis of unknown aetiology and is associated with a malignant neoplasm in 25% of cases. Herein, we report the case of a female patient in whom HM enabled us to diagnose gastric carcinoma. OBSERVATION: A 74-year-old woman consulted for papules and nodules on the hands associated with destructive polyarthralgia of the interphalangeal joints and asthenia. Biopsy of a nodule revealed infiltration of the superficial dermis by numerous multinucleated giant cells containing eosinophilic cytoplasm; immunohistochemical analysis showed positive staining for CD68, confirming the diagnosis of MH. Additional screening tests for malignancy enabled us to diagnose gastric carcinoma. The cutaneous lesions disappeared several weeks after gastrectomy and no recurrence of HM or malignancy was seen in the ensuing 7 years. DISCUSSION: Fewer than 200 cases of HM have been reported. This non-Langerhans proliferative histiocytosis is a rare systemic disorder of unknown aetiology characterized clinically by papules and nodules associated with destructive polyarthralgia, and histologically by dermal proliferation of histiocytic multinucleated giant cells of skin, mucous membrane and synovial membrane. Routine screening for neoplasia should be performed following the discovery of HM as an association is seen in 25% of cases. However, the two diseases do not generally progress in parallel. In our case, the rapid disappearance of lesions within a few weeks of surgery suggests that HM was a true paraneoplastic syndrome.

Multicentric reticulohistiocytosis and urologic carcinomas: a possible paraneoplastic association.

Multicentric reticulohistiocytosis (MR) is a rare non-Langerhans histiocytosis that is characterized by cutaneous nodules and severe destructive arthritis. Although 25-30% of reported cases have been associated with internal malignancies, the pathophysiology of MR is unknown. Herein, we report two cases of MR that were associated with urologic neoplasms. Because the tumor suppressor gene p53 may play a role in the biology of other histiocytoses, we investigated its p53 immunoexpression in these two cases. Both cases were positive immunohistochemically, but it remains to be seen whether this finding is truly important in the pathogenesis of MR associated with underlying visceral neoplasms.

Xanthogranulomas associated with hematologic malignancy in adulthood.

Although xanthogranulomatosis (XG), defined as multiple xanthogranulomas occurring simultaneously, was originally described in infants and children, a number of adult cases have been reported. Adult XG, which generally presents in the absence of hyperlipidemia, has many similarities to the childhood variant. Among the similarities are reports of the simultaneous development of XG and hematologic disorders. Herein we report a case of XG in a 45-year-old man with B-cell acute lymphoblastic leukemia and review the literature regarding the association of XG and hematologic disorders in adults. We propose that xanthogranulomas seen in children and adults bear many similarities, clinically and histopathologically, and share an association with hematologic malignancies.

Xanthogranulomatous pancreatic abscess secondary to acute pancreatitis: two case reports.

Xanthogranuloma is a rare type of inflammation and very few cases have been reported in the pancreas. We report two cases with xanthogranulomatous pancreatic abscess that followed acute pancreatitis. In both cases, multiple pseudocysts in the pancreatic tail were infected with several species of bacteria and Candida albicans. In one case, abdominal angiography revealed a hypoperfused pancreatic tail due to prior atherosclerotic obliteration of the celiac and superior mesenteric arteries. In the other case, the splenic artery was completely occluded by a transarterial embolization performed to treat an aneurysm that appeared in the course of pancreatitis. In both cases, distal pancreatectomy was performed as inflammation of the pancreatic tail was resistant to conventional antibiotic therapy, and pathologic examination revealed xanthogranulomatous inflammation around the pancreatic tail and spleen. Although the underlying pathogenesis is unclear, the prolonged infection and/or relative hypoxia induced by hypoperfusion are likely causative factors for the xanthogranulomatous changes in these pancreatic abscesses.

Multicentric reticulohistiocytosis: a proliferation of macrophages with tropism for skin and joints, part II.

In this second part of the review of multicentric reticulohistiocytosis, the authors discuss its association with other diseases, in particular, cancer, and laboratory and therapeutic aspects of this incapacitating and disfiguring disease. Histopathologic aspects are characteristic: dense mononuclear infiltrate with typical multinucleated cells that contain periodic acid-Schiff-positive and diastasis-resistant material, conferring a "ground glass" aspect when stained with hematoxylineosin.

Macrophage activation syndrome in juvenile idiopathic arthritis.

UNLABELLED: Macrophage activation syndrome (MAS) is a rare and potentially lethal complication of chronic rheumatic diseases of childhood, in particular of systemic-onset juvenile idiopathic arthritis (s-JIA), resulting from uncontrolled activation and proliferation of T lymphocytes and macrophages. The onset, acute and dramatic, may mimic a flare of the underlying disease or a severe sepsis. Diagnosis is difficult and, until now, no specific criteria have been developed. Laboratory data show pancytopenia, coagulopathy, low ESR and low concentrations of serum albumin, and high levels of ferritin, liver enzymes and triglycerides. Activated macrophages are found in various organs, particularly in bone marrow. Most hypotheses on the mechanism underlying MAS are based on the data obtained in primary haemophagocytic lymphohistiocytosis (HLH), a genetic disease very similar to MAS. Prompt diagnosis is essential because prognosis is highly related to early treatment. The first approach was to use intravenous methylprednisolone pulse therapy; cyclosporin A was proposed in patients resistant to steroids. We describe nine patients affected by haemophagocytosis: seven patients developed MAS and two patients developed HLH. A child with s-JIA developed three episodes of MAS. After the third episode, as there was no improvement with pulses of methylprednisolone and cyclosporine, he was successfully given etanercept. CONCLUSION: Our data, together with a similar, published observation, suggest that the TNF inhibitor etanercept is potentially useful for obtaining remission in children not responding to steroids and cyclosporin A.

Proliferating multicentric reticulohistiocytosis associated with papillary serous carcinoma of the endometrium.

Multicentric reticulohistiocytosis (MRH) is a rare histocytic disease characterized by destructive arthritis in association with classic skin findings. Although MRH is not strictly a paraneoplastic disease, one quarter of cases are malignancy related. We report a case of MRH with an initial remission followed by an acute exacerbation several years later heralding the clinical presentation of endometrial carcinoma. During this flareup a skin biopsy specimen revealed a diffuse dermal infiltrate composed of histiocytes with ground-glass cytoplasm and multiple atypical mitoses. Approximately 40% of the cells stained with the proliferation marker Ki-67. Treatment of endometrial carcinoma resulted in improvement of skin and joint symptoms, and a repeat biopsy specimen no longer demonstrated mitotic figures. These findings support a reactive and proliferative cause of MRH.

Successful treatment of acute hepatitis A-associated hemophagocytic syndrome by intravenous immunoglobulin.

Virus-associated hemophagocytic syndrome (VAHS) is a rare complication in acute hepatitis A virus (HAV) infection. There is no standard therapy for VAHS and the clinical course is variable. Data on the use of intravenous immunoglobulin (IVIG) in the treatment of HAV-associated VAHS is limited. We report a previously healthy, 32-year-old man who presented with general malaise, fever, chills and splenomegaly. Laboratory examination showed marked elevation of aminotransferase, leukopenia and thrombocytopenia. Acute hepatitis A was documented by the presence of immunoglobulin M anti-HAV and compatible liver biopsy findings. Bone marrow examination revealed extensive hemophagocytosis. IVIG was administered after the diagnosis of HAV-associated VAHS. His symptoms and laboratory abnormalities improved, and following a smooth recovery he was discharged 1 month after admission.