Assuntos
Hepatite C/patologia , Histiocitose de Células não Langerhans/patologia , Dermatopatias/patologia , Xantomatose/patologia , Derme/fisiologia , Hepatite C/complicações , Histiocitose de Células não Langerhans/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Raras , Dermatopatias/etiologia , Xantomatose/etiologiaAssuntos
Humanos , Masculino , Dermatopatias/patologia , Xantomatose/patologia , Histiocitose de Células não Langerhans/patologia , Hepatite C/patologia , Dermatopatias/etiologia , Xantomatose/etiologia , Histiocitose de Células não Langerhans/etiologia , Hepatite C/complicações , Derme/fisiologia , Doenças Raras , Pessoa de Meia-IdadeAssuntos
Neoplasias da Mama/complicações , Histiocitose de Células não Langerhans/patologia , Síndromes Paraneoplásicas/patologia , Pele/patologia , Neoplasias Abdominais/secundário , Neoplasias da Mama/patologia , Feminino , Histiocitose de Células não Langerhans/etiologia , Humanos , Pessoa de Meia-Idade , RecidivaRESUMO
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.
Assuntos
Adenocarcinoma/complicações , Neoplasias das Tubas Uterinas/complicações , Histiocitose de Células não Langerhans/etiologia , Adenocarcinoma/diagnóstico , Adenocarcinoma/tratamento farmacológico , Adenocarcinoma/cirurgia , Antineoplásicos/uso terapêutico , Neoplasias das Tubas Uterinas/diagnóstico , Neoplasias das Tubas Uterinas/tratamento farmacológico , Neoplasias das Tubas Uterinas/cirurgia , Feminino , Mãos/patologia , Histiocitose de Células não Langerhans/diagnóstico , Histiocitose de Células não Langerhans/tratamento farmacológico , Histiocitose de Células não Langerhans/patologia , Humanos , Pessoa de Meia-Idade , Doenças RarasAssuntos
Histiocitose de Células não Langerhans/diagnóstico , Neoplasias Primárias Múltiplas/complicações , Dermatopatias/diagnóstico , Xantomatose/diagnóstico , Carcinoma de Células Escamosas/complicações , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/cirurgia , Feminino , Histiocitose de Células não Langerhans/etiologia , Histiocitose de Células não Langerhans/patologia , Humanos , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/diagnóstico , Neoplasias Primárias Múltiplas/patologia , Dermatopatias/etiologia , Dermatopatias/patologia , Neoplasias do Timo/complicações , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/patologia , Neoplasias do Timo/cirurgia , Câncer Papilífero da Tireoide/complicações , Câncer Papilífero da Tireoide/diagnóstico , Câncer Papilífero da Tireoide/patologia , Câncer Papilífero da Tireoide/cirurgia , Neoplasias da Glândula Tireoide/complicações , Neoplasias da Glândula Tireoide/diagnóstico , Neoplasias da Glândula Tireoide/patologia , Neoplasias da Glândula Tireoide/cirurgia , Resultado do Tratamento , Xantomatose/etiologia , Xantomatose/patologiaAssuntos
Histiocitose de Células não Langerhans/etiologia , Síndromes Paraneoplásicas/etiologia , Timoma/complicações , Neoplasias do Timo/complicações , Idoso , Histiocitose de Células não Langerhans/diagnóstico por imagem , Humanos , Masculino , Síndromes Paraneoplásicas/diagnóstico por imagem , Radiografia , Timoma/diagnóstico por imagem , Neoplasias do Timo/diagnóstico por imagem , Tomografia Computadorizada por Raios XAssuntos
Proteínas Estimuladoras de Ligação a CCAAT/genética , Histiocitose de Células não Langerhans/etiologia , Fator de Transcrição PAX5/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Criança , Histiocitose de Células não Langerhans/patologia , Humanos , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Prognóstico , RNA Mensageiro/genética , Reação em Cadeia da Polimerase em Tempo Real , Reação em Cadeia da Polimerase Via Transcriptase ReversaAssuntos
Neoplasias da Mama/complicações , Dermatoses Faciais/etiologia , Dermatoses da Mão/etiologia , Histiocitose de Células não Langerhans/etiologia , Adulto , Biópsia , Dermatoses Faciais/patologia , Feminino , Dermatoses da Mão/diagnóstico por imagem , Dermatoses da Mão/patologia , Histiocitose de Células não Langerhans/diagnóstico por imagem , Histiocitose de Células não Langerhans/patologia , Humanos , Pescoço , CintilografiaRESUMO
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.
Assuntos
Artroplastia do Joelho/efeitos adversos , Histiocitose de Células não Langerhans , Complicações Pós-Operatórias , Dermatopatias , Idoso , Antígenos CD/metabolismo , Antígenos de Diferenciação Mielomonocítica/metabolismo , Feminino , Histiocitose de Células não Langerhans/etiologia , Histiocitose de Células não Langerhans/metabolismo , Histiocitose de Células não Langerhans/patologia , Humanos , Complicações Pós-Operatórias/metabolismo , Complicações Pós-Operatórias/patologia , Dermatopatias/etiologia , Dermatopatias/metabolismo , Dermatopatias/patologiaRESUMO
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.
Assuntos
Adenocarcinoma/complicações , Artrite/etiologia , Dermatoses da Mão/etiologia , Histiocitose de Células não Langerhans/etiologia , Segunda Neoplasia Primária/complicações , Síndromes Paraneoplásicas/etiologia , Neoplasias Gástricas/complicações , Adenocarcinoma/cirurgia , Idoso , Derme/patologia , Feminino , Gastrectomia , Células Gigantes/patologia , Dermatoses da Mão/diagnóstico , Dermatoses da Mão/patologia , Histiócitos/patologia , Histiocitose de Células não Langerhans/diagnóstico , Histiocitose de Células não Langerhans/patologia , Humanos , Segunda Neoplasia Primária/cirurgia , Síndromes Paraneoplásicas/diagnóstico , Indução de Remissão , Neoplasias Gástricas/cirurgia , Neoplasias do Colo do Útero/radioterapia , Neoplasias do Colo do Útero/cirurgia , Redução de PesoRESUMO
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.
Assuntos
Histiocitose de Células não Langerhans/patologia , Síndromes Paraneoplásicas/patologia , Neoplasias Urológicas/complicações , Carcinoma Neuroendócrino/complicações , Carcinoma de Células Renais/patologia , Carcinoma de Células Pequenas/complicações , Feminino , Histiocitose de Células não Langerhans/etiologia , Histiocitose de Células não Langerhans/genética , Humanos , Imuno-Histoquímica , Neoplasias Renais/complicações , Masculino , Pessoa de Meia-Idade , Síndromes Paraneoplásicas/etiologia , Síndromes Paraneoplásicas/genética , Proteína Supressora de Tumor p53/biossíntese , Proteína Supressora de Tumor p53/genética , Neoplasias da Bexiga Urinária/complicaçõesRESUMO
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.
Assuntos
Neoplasias Hematológicas/complicações , Histiocitose de Células não Langerhans/etiologia , Leucemia de Células B/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Dermatopatias/etiologia , Idoso , Criança , Fator XIIIa/análise , Feminino , Histiocitose de Células não Langerhans/patologia , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Dermatopatias/patologia , Xantogranuloma Juvenil/etiologia , Xantogranuloma Juvenil/patologiaRESUMO
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.
Assuntos
Histiocitose de Células não Langerhans/diagnóstico , Histiocitose de Células não Langerhans/etiologia , Pancreatite/diagnóstico , Pancreatite/etiologia , Abscesso Abdominal/etiologia , Doença Aguda , Idoso , Aneurisma/cirurgia , Angiografia , Candida albicans/metabolismo , Humanos , Imageamento Tridimensional , Inflamação , Masculino , Pessoa de Meia-Idade , Pancreatectomia/métodos , Artéria Esplênica/patologia , Tomografia Computadorizada por Raios X/métodos , Resultado do TratamentoRESUMO
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.
Assuntos
Artrite/patologia , Osso e Ossos/patologia , Histiocitose de Células não Langerhans , Articulações/patologia , Pele/patologia , Antígenos CD/análise , Artrite/diagnóstico por imagem , Doenças Autoimunes/complicações , Osso e Ossos/diagnóstico por imagem , Proliferação de Células , Histiócitos/metabolismo , Histiocitose de Células não Langerhans/etiologia , Histiocitose de Células não Langerhans/patologia , Histiocitose de Células não Langerhans/terapia , Humanos , Microscopia Eletrônica , Neoplasias/complicações , RadiografiaRESUMO
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.
Assuntos
Artrite Juvenil/complicações , Histiocitose de Células não Langerhans , Ativação de Macrófagos , Anti-Inflamatórios/administração & dosagem , Antirreumáticos/uso terapêutico , Artrite Juvenil/imunologia , Artrite Juvenil/fisiopatologia , Criança , Ciclosporina/uso terapêutico , Feminino , Histiocitose de Células não Langerhans/diagnóstico , Histiocitose de Células não Langerhans/tratamento farmacológico , Histiocitose de Células não Langerhans/etiologia , Histiocitose de Células não Langerhans/genética , Humanos , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/genética , Ativação de Macrófagos/imunologia , Masculino , Metilprednisolona/administração & dosagem , Pulsoterapia , SíndromeRESUMO
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.
Assuntos
Cistadenoma Papilar/complicações , Neoplasias do Endométrio/complicações , Histiocitose de Células não Langerhans/etiologia , Idoso , Feminino , Histiocitose de Células não Langerhans/patologia , HumanosRESUMO
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.