Langerhans cell histiocytosis of liver

Langerhans cell histiocytosis (LCH) is a rare disorder of unknown etiology caused by proliferation of Langerhans cells. It can involve single organ system to multi organ systems and clinical presentation is variable depending on the organ involved and have different prognosis. LCH is common in children when compared to adults. Hepatic involvement in adults is relatively rare. Liver involvement has considerable impact on survival rates. Histopathology and immunohistochemistry provide the definitive diagnosis. Authors report a case of Langerhans cell histiocytosis in a young adult with hepatic involvement.

T cell/histiocyte rich large B cell lymphoma of ascending colon: a rare entity

Colonic T cell/histiocyte rich large B cell lymphoma (THRLBCL) is a very unusual occurrence never described before. A 41-year anaemic male presented with loss of weight and appetite for 7 months and fever with Malena for 1 month. Abdominal examination revealed a 4×6 cm retroperitoneal lump in the right iliac fossa. Radiological investigations (USG and CECT whole abdomen) reported an asymmetrical ill-defined growth in ascending colon and caecum with loco-regional lymphadenopathy. Surgical exploration revealed an ascending colon mass with retroperitoneal lymphadenopathy. Right hemi-colectomy with end ileostomy was done and specimen was sent for histopathology which diagnosed it to be a case of THRLBCL of colon. Patient was followed up after 2 weeks and was planned for chemotherapy.

Histiocytic necrotizing lymphadenitis characterized by fever and joint pain:a case report / 中国感染控制杂志

A 27-year-old woman was admitted to a hospital due to intermittent fever for 20+ days and swelling pain in knee joint for 7 days.Patients was with persistent fever,the highest temperature to 40.1°C,the double knee joint swelling pain,a large number of scattered red needle-like rash were visible on bilateral hands and legs,lymph-adenectasis,splenectasis,bilateral hands interphalangeal joint and knee joint swelling pain,lymph nodes were con-fined to the bilateral axillary with soft texture,local tenderness,and good activity.Pathological results of left axil-lary lymph node showed the following finding:fragmental lymph node tissue with incomplete structure,lymphoid follicles existed,there were diffuse histocytes,immunoblasts,and a few small lymphocytes proliferation in the re-duced or disappeared area of follicular,caryokinesis could be easily seen,there were patches of necrosis foci with varied size,nucleus disintegrated,fibrinoid necrosis blood vessel with bleeding could be seen.Immunohistochemical detection showed the following results:CD3 (diffuse +);CD20 (follicular +);CD79α(follicular +);CD68 (+);EMA (-);ALK (-);CD15 (a small amount of cells +);CD30 (+),CD4 (-),CD5 (+);CD10 (-);bcl-2 (+);CD21 (+);telomerase B (-);TIA-1 (-);EBV(-)Ki-67 (+).Through a combination of clinical and immunohistochemical detection results,the diagnosis of histiocytic necrotizing lymphadenitis was made.Patient discontinued antimicrobial drugs,after she was treated with 5% glucose 100 mL + hydrocortisone sodium succinate 200 mg intravenous drip for 3 days,patients had no fever,axillary lymph node gradually dwindled.Then patients was treated with methylprednisolone 8 mg/d,twice a day,reduced 1 tablet every two weeks,and stopped eventual-ly.Patients was followed up for two years,repeated examination of blood routine,liver and kidney function were in the normal range,bilateral knee didn’t swell,could walk freely,there appeared no enlargement of lymph node.

Histiocitoma de Células de Langerhans en canino: reporte de caso en Colombia / Langerhans Cell Histiocytoma in canine: a case report in Colombia / Histiocitoma das células de Langerhans em caninos: relato de caso na Colômbia

Resumen Se reporta el caso clínico de un paciente canino de 10 años de edad, macho, mestizo y esterilizado, que llega a la Clínica veterinaria de Antioquia, Medellín Colombia, con una masa de 5 cms de diámetro en la región lateral del prepucio. La masa de forma circular y de consistencia firme, a la evaluación histopatológica correspondió a un histiocitoma de las células de Langerhans. El paciente fue sometido a resección quirúrgica de la masa y tratado posteriormente con cefalexina 25 mg/kg cada 12 horas oral por 1 semana, prednisolona por 20 días y desinfección de la zona con clorhexidina cada 12 horas, además se aplica en la zona dimetilsulfóxido tópico cada 12 horas por 3 semanas. Debido a lo poco descrito este tipo de histiocitoma en dermatología, se considera de valor científico su reporte.

Abstract A 10–year-old crossbred neutered dog was submitted to Clínica Veterinaria de Antioquia (Medellín, Colombia) presenting a firm and circular mass of 5 cm in diameter in the lateral region of the foreskin. After histopathological evaluation, the mass corresponded to a Langerhans cell histiocytoma. The patient underwent surgical resection of the mass and was then treated with cephalexin (25 mg/kg every 12 hours, orally, for 1 week), prednisolone (20 days), disinfection of the area (every 12 hours using chlorhexidine), and applying dimethyl sulfoxide on the area (every 12 hours for 3 weeks). We consider this report has a scientific value because this type of histiocytoma is rarely described in dermatology.

Resumo Relatar o caso clínico de um paciente canino de 10 anos de idade, macho, mestiço e esterilizado, que chegou a Clínica Veterinária de Antioquia, Medellín, Colômbia, com uma massa de 5 cm de diâmetro na região lateral do prepúcio, a massa era de forma circular e de consistência firme, na avaliação histopatológica revelou-se um histiocitoma das células de Langerhans, o paciente foi submetido a resseção cirúrgica da massa e tratado posteriormente com cefalexina 25 mg/kg a cada 12 horas oral durante uma semana, prednisolona por 20 dias e desinfecção da região com clorexidina a cada 12 horas, além disto, se aplicou na região afetada dimetilsulfóxido tópico a cada 12 horas durante três semanas. Devido as poucas referencias escritas deste tipo de histiocitoma em dermatologia, considera-se de valor cientifico seu relato.

Infection Associated Hemophagocytic Lymphohistiocytosis: A Case Report.

The term hemophagocytosis describes the pathologic finding of activated macrophages, engulfing erythrocytes, leukocytes, platelets and their precursor cells. This phenomenon is an important finding in patients with hemophagocytic syndrome, more properly referred to as hemophagocytic lymphohistiocytosis (HLH). HLH is a distinct clinical entity characterized by fever, pancytopenia, splenomegaly and hemophagocytosis in bone marrow, liver or lymph nodes. It has been associated with a variety of viral, bacterial, fungal and parasitic infections, as well as collagen-vascular diseases and malignancies and is uniformly fatal if left untreated. We report Staphylococcal aureus-induced hemophagocytic lymphohistiocytosis in a 3-month-old girl presenting with respiratory distress, sepsis and multiorgan failure. This case report may at least in part guide pediatricians and other physicians to recognize this rare entity of infection triggering fatal HLH and thus proper treatment may be instituted in those affected with this disease at the earliest.

Characterization of the Proliferated Histiocytes in Acute Leukemia by Performing Immunohistochemistry

BACKGROUND: The bone marrow biopsy sections of acute leukemia patients occasionally reveal a proliferation of large mononuclear cells that accompany the leukemic blasts, and this proliferation shows a starry sky pattern. We characterized these large mononuclear cells by performing immunohistochemistry with 12 different antibodies. The clinical characteristics were examined and then we determined their difference from hemophagocytic lymphohistiocytosis (HLH) and malignant histiocytic disorders. METHODS: Of the 200 acute leukemic bone marrow biopsy samples, 11 ALL and 10 AML cases showed large mononuclear cell proliferations. The panel of antibodies used for immunohistochemistry included those against the mononuclear phagocyte system, and immunohistochemistry was performed on the patients' initial specimens and the complete remission specimens. 10 normal specimens, 4 initial CML specimens and their complete hematologic response specimens were included as controls. RESULTS: The large mononuclear cells showed immunohistochemical results consistent with histiocytes. They were negative for the markers of dendritic cells the histiocytes and cytokines that are involved in the pathogenesis of HLH and vascular proliferation. Histiocyte proliferation was not observed in the complete remission specimens and in the initial and complete hematological response specimens of the CML patients and the normal bone marrow specimens. None of the cases fulfilled the criteria of HLH, and all 5 ALL cases, for which the immunophenotype results were available, showed a B cell phenotype. CONCLUSION: We characterized the large mononuclear cell proliferations as reactive histiocyte proliferations and we differentiated these from those of secondary HLH and malignant histiocytic disorders. A proportion of the large mononuclear cells showed negative results for all 12 antibodies and they showed characteristics that were suggestive of small fat cells. The pathophysiology and the prognostic effect of the reactive histiocyte proliferation accompanying acute leukemia require further study.

Primary Cutaneous T-cell/histiocyte-rich B-cell Lymphoma / 대한피부과학회지

T-cell/histiocyte-rich B-cell lymphoma is a recently-described morphologic variant of diffuse large B-cell lymphoma which can be confused with nodular lymphocyte-predominant Hodgkin's lymphoma and reactive lymphadenopathies. T-cell/histiocyte-rich B-cell lymphoma is characterized by a few large, atypical B-lymphoid cells set in a background containing numerous reactive small T-cells and non-epithelioid histiocytes. This lymphoma follows a natural history similar to those of traditional diffuse large B-cell lymphoma. Although rare, the primary cutaneous form appears to have a better prognosis than its nodal counterpart. We report on a case of a 52-year-old woman with primary cutaneous T-cell/ histiocyte-rich B-cell lymphoma. To our knowledge, this is the first case of T-cell/histiocyte-rich B-cell lymphoma, with or without skin involvement, reported in the Korean literature.

A Case of Erdheim-Chester Disease with periodic fever and knee pain / 대한내과학회지

ECD is a rare, non-Langerhans form of histiocytosis. The first case was reported in 1930 by Chester and the term ECD was first described by Jaffe in 1972. Many authors have suggested that ECD is a part of the spectrum of Langerhans cell histiocytosis(LCH), whereas others believe ECD as a distinct lipoidosis because of the symmetric distribution of the long bone lesions on the roentgenogram and the lack of Langerhans granules and S-100 antibody staining in the foamy histiocytes. We experienced a case of Erdheim-Chester disease (ECD) with periodic night fever, myalgia and both knee pain, the first case reported in Korea.

Histiocytosis X Involving Orbital Soft Tissue

Histiocytosis X is an uncommon multisystemic disease of unknown etiology which is characterized by the accumulation of histiocytes in various tissues, and variable clinical course. In patients with histiocytosis X, orbital involvement is common and has a distinct prediction for the superotemporal orbital bone. Orbital involvement is almost invariably associated with a osteolytic lesion of the orbital wall. Disease confined to orbital soft tissues without bone involvement is rare. We report the clinicopathologic features of an unusual form of histiocytosis X in a 2-year-old female who showed a rapidly growing lower eyelid mass. No other ocular or extraocular involvement was detected. The mass was excised and diagnosis was confirmed by hematoxylin eosin staining, which showed large histiocytes and a number of eosinophils, plasma cells and lymphocytes. In addition, the histiocytes were immunohistochemically stain for s-100 protein.

Ethical View in Human Stem cell Research / 中国医学伦理学

This article discussed on the ethical problems in human stem cell research.It asserted:The essence of its ethical quarrel in stem histiocyte research is the social impartiality in the distribution of scarce health resources,while the sticking point of the human crisis in embryonic stem cell research is the problems on the status and right for the embryos.It pointed out that the ethical principles of life sciences shand be newly constituted.

Distribution of S-100 Protein Positive Dendritic Cell and its Correlation with Degree of Malignancy in Gastric Carcinoma

The present study was performed in order to investigate the correlation among the number of T zone histiocytes, the clinicopathologic parameters and the patient's survival. The subjects in this study were 131 advanced gastric carcinomas including 86 follow-up cases and 41 early gastric carcinomas. The infiltration of T zone histiocytes into tumor tissues was investigated using the immunohistochemical method with andti s-100 protein, nd the data obtained were evaluated statistically. T zone histiocytes were scattered among the cancer cells and in areas formed clusters within the cancer stroma. These cells were abundantly present in tumor tissues and around lymphoid follicles but were rarely seen in surrounding normal stomach tissue. Analysis of the number of T zone histiocytes showed no correlation between the number of T zone histiocytes and the clinicopathologic features except the degree of lymphocytic infiltration within the advanced and the early gastric carcinomas. Survival of the patients with a marked infiltration of T zone histiocytes in stage III was longer than that with mild infiltration of T zone histiocytes, but there was no statistical significance(P value=0.084). Multivariate regressio analysis revealed that the depth of invasion(P value=0.0074) and the lymphocytic infiltration(P value=0.0152) were the important prognostic factors. The results indicate that good prognosis is expected in cases with less deep invasion and high lymphocytic infiltration, and that the number of T zone histiocytes is in proportio to the degree of lymphocyte infiltration within the tumor, although not directly correlated with the patient's survival.

Kupffer Cells in Hepatocellular Carcinoma

Kupffer cells are tissue macrophages (histiocytes) fixed in hepatie sinusoids. Since malignant hepatocytes are the only tumor parencymal cells of the hepatocellular carcinoma, theoretically there are no Kupffer cells within the hepatocellular carcinoma. To clarify whether it is true or not, 12 cases of hepatocellular carcinoma of the trabecular type with some extents of the non-neoplastic surrounding liver were subjected to immunoperoxidase staining for lysozyme and S-100 protein and the results are as follows. 1) Kupffer cells were stained positively by the immunoperoxidase staining for lysozyme but not for S-100 protein, indicating that they are monocyte derived macrophages. 2) Kupffer cells were also present within the hepatocellular carcinoma, but were 2-7 times fewer within the hepatocellular carcinoma than in the non-neoplastic areas (p<0.05). 3) The non-neoplastic hepatic tissue of patients with serum HBsAg shows a tendency to have more kupffer cells than those without HBsAg.

An Immunohistochemical Study of Proliferative Disorders of Histiocytes

ln an attempt to clarify the dual origin histiocytes and to reclassify histiocytic proliferative disorders according to their immunohistochemical properties, normal histiocytes and histiocytes in selected proliferative disorders were stained using the peroxidase-antiperoxidase method for lysozyme, 1-antichymotrypsin and for S-100 protein. The proliferated histocytes of cosinophilic granutoma and Letterer-siwe disease were strongly immunoreactive for S-100 protein. In histiocytic medullary reticulosis (HMR) and in histiocytic lymphoma, all three markers were found within the tumor cells. ln fibrous histiocytoma and in juvenile xanthogranuloma, only a few weakly immunoreactive cells for S-100 protein were observed. lnflammatory malignant fibrous histiocytoma(MFH) (Xanthosarcoma) and xanthoma were immunoreactive for 1-antichymotrypsin and lysozyme respectively. ln MFH of the storiform -pleomorphic type and in atypical fibroxanthoma, stains using all of the histiocytic markers were negative. These results suggest that eosinophilic granuloma. Letterer-Siew disease, fibroxanthoma and juvenile xanthogranloma are proliferative disorder of T-zone histiocytes; HMR and histiocytic lymphoma are those of pluripotential stem cells capable of dual histiocytic differentiation; xanthoma and xanthosarcoma are monocytic proliferative disease; and MFH of the storiform-pleomorphic type and atypical fibroxanthoma are not true histiocytic diseases.

Histiocytic Medullary Reticulosis in Children: Report of Four Cases and Analysis of Previously Reported Cases

Histiocytic Medullary Reticulosis(H.M.R.) is a rapidly progressing fatal disease seen most often in adults, but it has been reported in children younger than 15 years of age. H.M.R. is clinically characterized by fever, wasting generalized lymphadenopathy and hepatosplenomegaly. In the terminal stage, jaundice, purpura, anemia and pancytopenia are all present with or without skin involvement. Cardinal pathologic features are systemized proliferation of atypical, neoplastic, erythrophagocytic histiocytes and their precursors throughout the lymphoreticular tissues. Four cases of H.M.R. occurring in pediatric age group, i.e., 5 years, 6 years, 12 years and 14 years of age, respectively, are presented with discussion of the clinico-pathologic characteristics and management. Cases 1,2 and 3 died within 6 months after the onset of illness. Case 1 of our series was particularly younger than in previously reported cases. This was the case that the diagnosis was confirmed by postmortem examination. Analysis of previously reported 25 cases occurring in ages younger than 20 years of age was done.