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1.
Autops. Case Rep ; 13: e2023439, 2023. graf
Article in English | LILACS-Express | LILACS | ID: biblio-1447436

ABSTRACT

ABSTRACT Xanthogranulomatous inflammation is a rare benign inflammatory lesion characterized by sheets of lipid-laden foamy histiocytes. It has been reported in various organs, mainly the kidney and gall bladder. Xanthogranulomatous endometritis (XGE) is sporadic, with only a few cases reported in the English medical literature. Herein, we report a case of xanthogranulomatous endometritis with the formation of stones in a 50-year-old female patient with a prolapsed uterus. Grossly the endometrium was irregular, and the uterine cavity was filled with a yellow friable material, a polypoid growth, and yellowish stones. The microscopy showed sheets of histiocytes with few preserved endometrial glands. In this case, the xanthogranulomatous inflammation may mimic a clear cell carcinoma involving the endometrium and myometrium. One of the important differential diagnoses is malakoplakia. Immunohistochemistry and special stains are helpful in diagnosis.

2.
J Cancer Res Ther ; 2020 Jul; 16(3): 665-667
Article | IMSEAR | ID: sea-213679

ABSTRACT

Histiocytic sarcoma (HS) is an extremely rare and aggressive hematopoietic tumor. Although it can be seen at any anatomic location, the most common primary sites are skin as extranodal region, locations including the lymph nodes and gastrointestinal tract. To the best of our knowledge, in light of PubMed search, this is the first primary tonsillar HS case presented with disseminated metastases at the time of diagnosis. A 58-year-old male patient applied with swelling on the right side of the neck, difficulty in swallowing, and weight loss. Positron emission tomography computed tomography was performed and increased pathological 18F fluorodeoxy D glucose uptake was detected in the right palatine tonsil, bilateral cervical multiple lymph nodes, liver masses, intra abdominal lymph nodes, and nodular lesion in the left adrenal gland. Tonsillectomy was performed and the pathological result was reported as HS. The patient did not respond to any treatment and had died after 5 months from the date of diagnosis. In conclusion, HS is generally diagnosed at advanced stage, it has limited chemotherapy response and high mortality rates. To understand this rare disease's pathophysiological and clinical features, further investigations are needed

3.
Autops. Case Rep ; 10(2): e2020158, Apr.-June 2020. tab, graf
Article in English | LILACS | ID: biblio-1131814

ABSTRACT

Giant cell reparative granuloma (GCRG) was first described in the 1950s. It is an uncommon and benign reactive tumor that is believed to occur after trauma or inflammation. It most commonly occurs in the maxilla and mandible and rarely affects the nasal cavity. It is often seen in children and during the second to third decades of life, predominantly seen among females. Histopathologically, GCRG shows many osteoclast-like multinucleated giant cells scattered in a background of mononuclear stromal cells and spindle-shaped fibroblasts also associated with areas of hemorrhage. The distinction between GCRG and giant cell tumors (GCT)is crucial since both have a similar clinical and histological presentation, but both have different management. GCTs have malignant potential, may metastasize, and have a high rate of recurrence. Surgical excision is the mainstay therapy of GCRG to ensure a low rate of recurrence. Here we discuss two cases GCRG, both presenting as nasal mass.


Subject(s)
Humans , Female , Adolescent , Adult , Giant Cells , Granuloma/pathology , Nasal Cavity , Diagnosis, Differential , Giant Cell Tumors
4.
Indian J Lepr ; 2019 Jun; 91(2): 159-164
Article | IMSEAR | ID: sea-195044

ABSTRACT

Type II Lepra reaction is a Th2 mediated hypersensitivity reaction characterized by involvement of skin and nerves. Herein we report an unusual leprosy case that had a rheumatic mode of onset of Type II Lepra reaction. This case also had livedoid plaques, ulcers on the genitalia, extremities as the initial manifestation of Hansen's Disease and atypical histopathology. His symptoms had started 1 year back as polyarthralgia of both knees and small joints of hands and feet for which he was on immunosuppressant by a Rheumatologist who made a diagnosis of rheumatoid arthritis. After 6 months, patient developed red painful lesions; initially on arms, chest and trunk followed by legs. Subsequently, painful ulcerations developed on feet, knees and genitalia and a dermatology referral was made. Histopathology and slit skin smears led to diagnosis of Lepromatous leprosy in Type II Lepra reaction. Hansen's disease is a great imitator. Rheumatic manifestations can be the initial presentation of Type II Lepra reaction. Rheumatic manifestations thus can be the initial presentation of Type II Lepra reaction even before starting Multi drug therapy. High index of suspicion and routine slit skin smears in small peripheral settings can be helpful in diagnosing and treating such cases.

5.
Journal of Breast Cancer ; : 491-496, 2019.
Article in English | WPRIM | ID: wpr-764273

ABSTRACT

Histiocytic sarcoma is a rare hematologic malignancy, with very few cases of primary histiocytic sarcoma of the breast described in English scientific literature. Herein, we describe a case of primary histiocytic sarcoma of the breast in a 75-year-old woman, with no clinical history of malignant tumors, who presented with a palpable solitary breast mass. Microscopically, the resected breast mass showed large pleomorphic cells, some multinucleated giant cells, and admixed inflammatory components. The pleomorphic tumor cells further showed a diffuse, noncohesive growth pattern, an abundant eosinophilic cytoplasm, and strong and diffuse immunoreactivity for cluster of differentiation (CD) 68 and CD163. Furthermore, a whole-body positron-emission tomography/computed tomography using deoxy-2-[¹⁸F]fluoro-D-glucose performed after surgery showed no other masses or lesions. After surgical excision, the patient was followed up, and no evidence of tumor recurrence or metastasis was noted.


Subject(s)
Aged , Female , Humans , Breast , Cytoplasm , Eosinophils , Giant Cells , Hematologic Neoplasms , Histiocytes , Histiocytic Sarcoma , Neoplasm Metastasis , Recurrence
6.
An. bras. dermatol ; 93(5): 740-742, Sept.-Oct. 2018. graf
Article in English | LILACS | ID: biblio-949950

ABSTRACT

Abstract: Light microscopy of granuloma annulare shows mucin deposition with lympho-histiocytic infiltrate. We describe the ultrastructural three-dimensional aspects of a typical case of granuloma with characteristic histopathological findings. At the ultrastructural level, affected collagen bundles and granular mucin deposition were observed. Round cells corresponding to lymphocytes were identified. Bigger oval cells, corresponding to isolated or palisading histiocytes were also found. The ultrastructural aspects overlap with light microscopy and contribute to its iconographic documentation.


Subject(s)
Humans , Microscopy, Electron, Scanning , Collagen/ultrastructure , Granuloma Annulare/pathology , Histiocytes/ultrastructure , Mucins/metabolism
7.
An. bras. dermatol ; 93(4): 595-597, July-Aug. 2018. graf
Article in English | LILACS | ID: biblio-949910

ABSTRACT

Abstract: Reticulohistiocytomas represent a group of benign histiocytic dermal proliferations, which occur either sporadically as solitary cutaneous nodules or, when multiple, in association with systemic disease. Due to its nonspecific clinical presentation, reticulohistiocytoma may mimic other benign or malignant skin neoplasms; therefore, in most cases, a biopsy is needed in order to establish the correct diagnosis. The histology is typically characterized by the presence of large histiocytes with abundant eosinophilic cytoplasm with immunohistochemical profile positive for CD68, CD163, and vimentin. The authors report the case of a patient with solitary reticulohistiocytoma with illustrative clinical, dermoscopic, and histologic features.


Subject(s)
Female , Middle Aged , Toes/pathology , Histiocytosis, Non-Langerhans-Cell/diagnosis , Foot Dermatoses/diagnosis , Immunohistochemistry , Histiocytosis, Non-Langerhans-Cell/pathology , Dermoscopy , Diagnosis, Differential , Foot Dermatoses/pathology
8.
Rev. gastroenterol. Perú ; 38(3): 280-284, jul.-set. 2018. ilus, tab
Article in Spanish | LILACS | ID: biblio-1014095

ABSTRACT

La enfermedad de Gaucher, es un trastorno autosómico recesivo de depósito lisosomal que se caracteriza por deficiencia de la beta-glucocerebrosidasa que lleva a la acumulación de glucosilceramida principalmente en células del sistema fagocítico mononuclear causando afectaciones sistémicas. Se presenta paciente varón de 20 años que cursa con dolor crónico en hipocondrio izquierdo con episodios de sangrados desde hace 3 años y sensación de alza térmica, al examen físico se identificó ictericia y esplenomegalia masiva, sin afectación neurológica. Como apoyo al diagnóstico se mostró osteoporosis severa, pancitopenia y como hallazgo inesperado la presencia de trombosis de vena porta con transformación cavernomatosa complicada con biliopatía portal simulando un tumor de klatskin, los estudios de médula y enzimáticos eran compatibles con enfermedad de Gaucher, por lo cual recibió tratamiento con imiglucerasa realizando seguimiento. Es un caso poco frecuente, de gran interés, heterogeneidad en sus manifestaciones clínicas e inéditas por su complicación, constituyendo un desafío llegar a su diagnóstico de esta enfermedad huérfana.


Gaucher disease is an autosomal recessive lysosomal storage disorder characterized by deficiency of beta-glucosidase that would lead to the accumulation of glucosylceramide mainly in cells of the mononuclear phagocytic system causing systemic effectations. We present a patient of twenty years who is suffering from chronic pain in the left hypochondrium with episodes of bleeding for 3 years and sensation of thermal rise, physical examination revealed jaundice and massive splenomegaly, without neurological involvement. Severe osteoporosis, pancytopenia, and the presence of portal vein thrombosis with cavernomatous transformation complicated by portal biliopathy simulating a klatskin tumor, marrow and enzymatic studies were compatible with Gaucher disease, were shown as unexpected findings. he received treatment with imiglucerase, following up. It is a rare case, of great interest, heterogeneity in its clinical manifestations and unpublished by its complication, constituting a challenge to reach its diagnosis of this orphan disease.


Subject(s)
Humans , Male , Young Adult , Portal Vein/abnormalities , Portal Vein/pathology , Bile Duct Diseases/etiology , Gaucher Disease/complications , Hemangioma, Cavernous/complications , Gastrointestinal Hemorrhage/etiology , Hypertension, Portal/complications , Portal Vein/diagnostic imaging , Renal Veins/pathology , Renal Veins/diagnostic imaging , Splenectomy , Splenic Vein/pathology , Splenic Vein/diagnostic imaging , Bile Ducts, Intrahepatic/pathology , Bile Ducts, Intrahepatic/diagnostic imaging , Tomography, X-Ray Computed , Dilatation, Pathologic/etiology , Enzyme Replacement Therapy , Gallbladder/blood supply , Gaucher Disease/diagnosis , Gaucher Disease/drug therapy , Glucosylceramidase/therapeutic use , Hypertension, Portal/diagnostic imaging , Mesenteric Veins/pathology , Mesenteric Veins/diagnostic imaging
9.
Autops. Case Rep ; 8(1): e2018002, Jan.-Mar. 2018. ilus
Article in English | LILACS | ID: biblio-905467

ABSTRACT

Mesothelial/monocytic incidental cardiac excrescences (MICE) are unusual findings during the histological analysis of material from the pericardium, mediastinum, or other tissues collected in open-heart surgery. Despite their somewhat worrisome histological appearance, they show a benign clinical course, and further treatment is virtually never necessary. Hence, the importance of recognizing the entity relays in its differential diagnosis, as an unaware medical pathologist may misinterpret it for a malignant neoplasm. Other mesothelial and histiocytic proliferative lesions, sharing very close histological morphology and immunohistochemistry features with MICE, have been described in sites other than the heart or the mediastinum. This similarity has led to the proposal of the common denomination "histiocytosis with raisinoid nuclei." We report three cases from the pathology archives of the Heart Institute of São Paulo University (Incor/HC-FMUSP), diagnosed as "mesothelial/monocytic incidental cardiac excrescence," with immunohistochemical documentation, and provide a literature review of this entity.


Subject(s)
Humans , Male , Adult , Middle Aged , Aged , Heart Diseases/pathology , Histiocytes , Diagnosis, Differential , Epithelium/injuries , Incidental Findings
10.
Rev. argent. dermatol ; 99(1): 1-10, mar. 2018. ilus
Article in Spanish | LILACS | ID: biblio-897397

ABSTRACT

La lepra histioide de Wade, es una forma clínico-histopatológica especial de lepra multibacilar, caracterizada por la presencia de tubérculos (lesiones papulosas o nodulares) correspondientes a lepromas formados por histiocitos de morfología fusiforme. La misma es una variante infrecuente de lepra lepromatosa, que se desarrolla generalmente como recaída, en pacientes que recibieron monoterapia con dapsona, aunque se reportó su presentación de novo. En el presente trabajo, se comunica el caso clínico de una paciente de sexo femenino de 45 años de edad, con diagnóstico tardío de lepra histioide. Además, se examinan las características principales de esta forma particular de expresión de la lepra, sus diferencias con la forma clásica de presentación, los diagnósticos diferenciales que deben considerarse y la importancia de tener a esta patología entre las sospechas diagnósticas, para comenzar el tratamiento adecuado y evitar su propagación.


Wade's hystioid leprosy is a special clinical-pathological form of multibacillary leprosy, characterized by papular and nodular lepromas that consist of spindle histiocytes. It is a variant of lepromatous leprosy. Most of the cases have been related to dapsone resistance in the context of longterm monotherapy. De novo cases, not associated with previous anti-leprosy treatment, have been less frequently reported. This article presents the case of 45 years old female, with late diagnosis of histoid leprosy. There will be explained the main features of this particular form of leprosy, its differences with the classic presentation, the differential diagnoses to be considered, and the importance of having this pathology among diagnostic suspicions to begin adecuate treatment.

11.
Chinese Journal of Dermatology ; (12): 173-176, 2018.
Article in Chinese | WPRIM | ID: wpr-710352

ABSTRACT

Objective To investigate clinical manifestations,morphological characteristics of skin lesions,and histopathological features of cutaneous Rosai-Dorfman disease (CRDD).Methods Basic information and clinical data were collected from 20 patients with CRDD.According to the morphological characteristics,the skin lesions were classified into different types,and then subjected to histopathological examination and immunohistochemical staining.Results Of the 20 patients with CRDD,11 had multiple lesions,and 9 had solitary lesions.Skin lesions involved single anatomical site in 16 patients,multiple anatomical sites in 4 patients,and there were a total of 24 involved anatomical sites.Skin lesions on the 24 sites were divided into 3 main types,including papulonodular type (10/24,41.67%),infiltrating plaque type (12/24,50.00%) and tumor-like type (2/24,8.33%).Of the 20 patients,6 had mixed-type skin lesions,including 5 with papulonodular-type lesions complicated by infiltrating plaque-type lesions,and 1 with infiltrating plaque-type lesions complicated by tumor-like lesions.There were similar histopathological manifestations of skin lesions among the 24 involved anatomical sites.Concretely speaking,varying numbers of large histiocytes were scattered or distributed in sheets in the dermis and/or subcutaneous adipose tissue,with infiltration of plenty of inflammatory cells,mainly lymphocytes and plasma cells.Moreover,varying numbers of lymphocytes and neutrophils were observed in the cytoplasm of histiocytes.Immunohistochemically,these histiocytes were stained positive for S100 and CD68,but negative for CD1a.At 17 anatomical sites,lesions affected the full-thickness dermis,and the subcutaneous adipose tissues were involved at 13 of 17 sites.Of the 24 involved anatomical sites,lesions only affected the superficial to middle dermis at 6 sites,and affected the deep dermis and subcutaneous adipose tissue at 1 site.There were no obvious differences in the extent of lesion involvement and pattern of inflammatory infiltration among different morphological types of skin lesions.Conclusions CRDD mainly manifests as papulonodular-type and infiltrating plaque-type lesions,and tumor-like lesions are rare.Histopathologically,varying numbers of emperipoletic histiocytes can be observed in lesions of different types.

12.
Journal of University of Malaya Medical Centre ; : 1-3, 2018.
Article in English | WPRIM | ID: wpr-822783

ABSTRACT

@#Xanthogranulomatous cholecystitis is a rare histopathological finding and accounts for 1.3% to 5.2% of cases. It closely resembles gallbladder cancer because of its extensive inflammation and involvement of the surrounding organs. We are reporting a case where it presents as an extensive inflammatory mass mimicking gallbladder cancer.

13.
Rev. bras. ortop ; 52(3): 366-369, May.-June 2017. graf
Article in English | LILACS | ID: biblio-899148

ABSTRACT

ABSTRACT Hemosiderotic fibrohistiocytic lipomatous tumors are rare neoplasms that were first described in 2000. Initially considered a benign lipotamous lesion of the soft tissues, nowadays they are considered to be a locally aggressive tumor. They occur mainly in the foot and ankle of women in their fifth and sixth decades, although they may be found in any place in the lower limbs and, more rarely, in other parts of the body. Histologically, hemosiderotic fibrohistiocytic lipomatous tumors consist of a mixture of mature adipose tissue, fusiform cell fascicles, macrophages that often contain cytoplasmic hemosiderin, mononuclear inflammatory infiltrate, and stroma that may be focally myxoid. Local recurrence is observed in nearly one-third of all cases. There is no consensus in the literature whether this tumor is a part of a spectrum that comprises pleomorphic hyalinizing angiectatic tumors and myxoinflammatory fibroblastic malignant tumors, or if it is an independent entity. The authors report a case of a neoplasia after a diagnosis of a hemosiderotic fibrohistiocytic lipomatous tumor in a 38-year-old woman, with two recurrences and later sarcomatous transformation. An immunohistochemical study indicated myofibroblastic differentiation of a malignant neoplasm. To the best of the authors' knowledge, there are only few reported cases of malignant transformation in hemosiderotic fibrohistiocytic lipomatous tumors.


RESUMO Os tumores lipomatosos fibro-histiocíticos hemossideróticos são neoplasias raras que foram descritas pela primeira vez em 2000. Inicialmente considerada uma lesão lipomatosa benigna dos tecidos moles, atualmente é considerado um tumor localmente agressivo. Essas lesões ocorrem principalmente no pé e no tornozelo das mulheres na quinta e sexta décadas de vida, embora possam ser encontrados em qualquer lugar nos membros inferiores e, mais raramente, em outras partes do corpo. Histologicamente, tumores lipomatosos fibro-histiocíticos hemossideróticos são formados por uma combinação de tecido adiposo maduro, fascículos de células fusiformes, macrófagos que contêm frequentemente hemossiderina citoplasmática, infiltrado inflamatório mononuclear e estroma, que pode ser focalmente mixoide. Há recidiva local em quase um terço dos casos. Não existe consenso na literatura se esse tumor faz parte de um espectro que envolve os tumores angiectásicos hialinizantes pleomórficos e os tumores fibroblásticos mixoinflamatórios malignos ou se configura uma entidade independente. Relatamos um caso de uma neoplasia com diagnóstico inicial de tumor lipomatoso fibro-histiocítico hemossiderótico em uma mulher de 38 anos, com duas recorrências e posterior transformação sarcomatosa. O estudo imuno-histoquímico indicou diferenciação miofibroblástica da neoplasia maligna. Encontramos apenas poucos casos relatados de transformação maligna de tumor lipomatoso fibro-histiocítico hemossiderótico.


Subject(s)
Humans , Female , Adult , Adipose Tissue , Connective Tissue , Hemosiderin , Histiocytes , Sarcoma
14.
Braz. dent. sci ; 20(2): 152-158, 2017. ilus, tab
Article in English | LILACS, BBO | ID: biblio-846454

ABSTRACT

The fibrous histiocytoma is a soft tissue neoplasm that affects the dermis and the subcutaneous tissue, rarely is found in the oral cavity and perioral regions, and is originated from the proliferation of fibroblasts and histiocytes. The objective of this paper is to report a case of Benign Fibrous Histiocytoma in a 30-year-old male patient, complaining of a painless nodule in the tongue for about six months. With diagnostic clinical hypotheses of Fibrous Hyperplasia, Neurofibroma, Traumatic Neuroma, Fibrous Histiocytoma, Granular Cell Tumor or Ectomesenchymal Chondromyxoid Tumor a excisional biopsy was performed. The histopathological examination revealed a non-encapsulated proliferation of spindle cells with some giant multinucleated cells in the periphery of the lesion. Immunohistochemical reactions were performed, staining only for vimentin in the spindle cells and for CD68 in the multinucleated giant cells. According to these characteristics, the final diagnosis was Benign Fibrous Histiocytoma. The correct diagnosis of spindle shaped cell neoplasia must be performed with the aid of histopathological analysis and immunohistochemistry, mainly because the morphological similarities with other benign and malignant lesions. (AU)


O Histiocitoma Fibroso é uma neoplasia de partes moles que acomete a derme e o tecido subcutâneo, raramente é encontrado na cavidade oral e regiões periorais, e tem origem a partir da proliferação de fibroblastos ou histiócitos. O objetivo deste artigo é relatar um caso de Histiocitoma Fibroso Benigno em um paciente masculino, 30 anos de idade, com um nódulo indolor, bem delimitado, com duração de cerca de seis meses, localizado no dorso anterior da língua. Com as hipóteses clínicas diagnósticas de Hiperplasia Fibrosa, Neurofibroma, Neuroma Traumático, Histiocitoma Fibroso, Tumor de Células Granulares e Tumor Condromixoide Ectomesenquimal uma biópsia foi realizada sob anestesia local e a lesão foi fixada em formol a 10% e enviada para análise histopatológica. O exame histopatológico revelou uma proliferação não-encapsulada de células fusiformes com algumas células gigantes multinucleadas na periferia da lesão. A marcação imunohistoquímica foi positiva para CD68 nas células gigantes multinucleadas e para vimentina nas células fusiformes. O diagnóstico final foi de Histiocitoma Fibroso Benigno. Para um diagnóstico correto, este deve ser feito correlacionando características clínicas, análise histopatológica e imunohistoquímica devido à similaridade microscópica do Histiocitoma Fibroso com outras lesões com aspecto fusocelular, assim como similaridade clínica com outras lesões benignas e malignas.(AU)


Subject(s)
Humans , Male , Adult , Fibroblasts , Histiocytes , Histiocytoma, Benign Fibrous
15.
Indian J Pathol Microbiol ; 2016 Oct-Dec 59(4): 469-473
Article in English | IMSEAR | ID: sea-179639

ABSTRACT

Background: Bronchiectasis is a chronic disease characterized by permanent dilatation of the conducting airways accompanied by sustained inflammation. Aims: To assess whether chronic inflammation of lungs in bronchiectasis is associated with alterations in the numbers of infiltrating antigen presenting cell (APC). Setting and Design: Lobectomy specimens from 12 nonsmoker, nonasthmatic patients with acquired (noncongenital) bronchiectasis and six control patients were included in the study. Histopathology slides were reviewed, and immunohistochemical markers for dendritic cells (DCs) macrophages and Langerhans cells have been applied and analyzed. Materials and Methods: Tissue specimens were stained by immunohistochemistry using markers for DCs (CD83 and CD23), macrophages (CD68 and CD163), and Langerhans cells (CD1A and S‑100 protein). The mean cell counts of stained cells in five high power microscopic fields were recorded. Statistical Analysis Used: Descriptive statistics, mean, standard deviation, median, and interquartile range were used. A nonparametric Mann–Whitney U‑test was used to compare cell counts between bronchiectasis and control patients. P <0.05 was considered significant. Results: The mean age of patients with bronchiectasis and controls was 36.7 ± 16.6 and 31.8 ± 22.6 years, respectively. The predominant cell type among the patients was macrophage (median 50.5) followed by DCs (median 44.85), histiocytes (median 32), and Langerhans cells (median 5%). Compared to the controls a significantly higher number of macrophages (P = 0.01), DCs (P = 0.001), and Langerhans cells (P = 0.014) were present. Conclusion: Chronic inflammatory response in acquired (noncongenital) bronchiectasis is most probably mediated by increased infiltration of APCs in lung tissues.

16.
Article in English | IMSEAR | ID: sea-166345

ABSTRACT

Chediak higashi Syndrome (CHS) is a rare autosomal recessive multisystem disorder with a defect in granule morphogenesis with giant lysosomes in leucocyte and other cells. CHS is a rare disease, approximately 200 cases have been reported so far. It was described in detail by Chediak in 1952 and Higashi in 1954. 1½ year old male child presented with multiple hypopigment patches on lower extremities, light colored hair, Hepatosplenomegaly and generalised Lymphadenopathy. PBS shows giant prominent liliac to purple granules in neutrophils, band forms, few lymphocytes and monocytes. Bone marrow is hypercellular showing giant prominent gray blue to purple heterogeneous granules often multiple seen in many myeloid precursors, Neutrophils, few lymphocytes and monocytes. Occasional lymphocytes shows single giant liliac inclusions. Erythropoiesis, myeloid series and Megakaryocytes are mildly increased. Hemophagocytosis noted. CHS is characterised by partial oculocutaneous albinism, frequent fatal bacterial infections, bleeding diathesis and peripheral + Cranial nerve palsies. This disorder further culminates into accelerated phase (Lymphoproliferative Syndrome) progressing into pancytopenia. CHS is due to single gene mutation in LYST (CHS) gene localized to 1q chromosome. The diagnostic hallmark of CHS is presence of giant purple to blue violet inclusions in leucocytes. In this study granules are more prominent in Bone marrow than in PBS correlating well with previous studies. Approximately 85% of the cases, of CHS culminates into Accelerated phase showing Lymphohistiocytic infiltration progressing to pancytopenia and death due to infection. The very rare nature of this disease and its grave prognosis merits its reporting.

17.
Article in English | IMSEAR | ID: sea-157758

ABSTRACT

Juan-Ron fever named after Jaun Rosai and Ronald Dorfman is the fever associated with Rosai Dorfman disease also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML). It is a rare disorder of unknown etiology that is characterized by abundant histiocytes in the lymph nodes throughout the body. Usually patient presents with painless lymphadenopathy. We present a case of a 45 year old male who presented to us with bilateral cervical lymphadenopathy and fever, later on diagnosed to have SHML.

18.
Clinical Endoscopy ; : 358-361, 2014.
Article in English | WPRIM | ID: wpr-47278

ABSTRACT

Xanthoma is an uncommon nonneoplastic lesion resulting from the accumulation of histiocytes. It predominantly shows cutaneous manifestations associated with dyslipidemia. However, xanthoma of the esophagus is extremely rare. To the best of our knowledge, only 14 cases have been reported thus far. The clinical significance of this lesion has not been established. However, this lesion should be distinguished grossly from ectopic sebaceous glands and small subepithelial tumors such as carcinoid and granular cell tumor. Moreover, signet ring cell carcinoma, which contains round cells with abundant cytoplasm and has similar histologic features to xanthoma, should be distinguished microscopically.


Subject(s)
Carcinoid Tumor , Carcinoma, Signet Ring Cell , Cytoplasm , Dyslipidemias , Endoscopy , Esophagus , Granular Cell Tumor , Histiocytes , Sebaceous Glands , Xanthomatosis
19.
An. bras. dermatol ; 86(4,supl.1): 78-81, jul,-ago. 2011. ilus
Article in Portuguese | LILACS | ID: lil-604127

ABSTRACT

A histiocitose de células de Langerhans é representante de um raro grupo de síndromes histiocitárias, sendo caracterizada pela proliferação das células de Langerhans. Suas manifestações variam de lesão solitária a envolvimento multissistêmico, sendo o acometimento vulvar incomum. Segue-se o relato de caso refratário da doença limitada à pele, em mulher de 57 anos. A paciente apresentava história de pápulas eritematosas ulceradas em couro cabeludo, face, vulva, tronco e axila há seis anos. O diagnóstico da doença é difícil, sendo confirmado neste caso através de estudo imuno-histoquímico e se obteve resposta terapêutica e eficaz, com a administração de talidomida.


Langerhans cell histiocytosis is a member of a group of rare histiocytic syndromes and is characterized for the proliferation of histiocytes called Langerhans'cells. Its manifestations vary from a solitary injury to systemic involvement, and vulvar lesions are uncommon. We describe a refractory case of cutaneous limited disease in a 57-year-old woman. She presented with a 6-year history of an erythematous papular eruption of the scalp, face, vulva, trunk and axillae. The diagnosis is difficult and in this case it was confirmed through immunohistochemical study and clinical improvement was achieved with thalidomide.


Subject(s)
Female , Humans , Middle Aged , Histiocytosis, Langerhans-Cell/drug therapy , Immunosuppressive Agents/therapeutic use , Skin Diseases/drug therapy , Thalidomide/therapeutic use , Vulvar Diseases/drug therapy , Facial Dermatoses/drug therapy , Facial Dermatoses/pathology , Histiocytosis, Langerhans-Cell/pathology , Scalp Dermatoses/drug therapy , Scalp Dermatoses/pathology , Skin Diseases/pathology , Vulvar Diseases/pathology
20.
An. bras. dermatol ; 85(2): 211-215, mar.-abr. 2010. ilus
Article in Portuguese | LILACS | ID: lil-547479

ABSTRACT

O histiocitoma fibroso é tumor heterogéneo composto por fibroblastos, histiócitos e vasos sanguíneos. Efectuamos uma revisão histopatológica retrospectiva de 95 biopsias de histiocitomas fibrosos do nosso arquivo dos últimos 3,5 anos, com o objectivo de avaliar a localização, delimitação, alteração da epiderme, indução folículo-sebácea, celularidade, vascularização, padrão do colagénio e tipo de células constituintes. Na maioria das biopsias, confirmamos as características histopatológicas clássicas dos histiocitomas fibrosos. Achados interessantes observados no nosso estudo foram presença de células nódulos linfoides, mastócitos e infiltrado de células inflamatórias.


Fibrous histiocytoma (FH) is a heterogeneous tumor composed of fibroblasts, histiocytes, and blood vessels. We conducted a retrospective histopathologic analysis of 95 biopsies, performed over the last 3.5 years, of fibrous histiocytomas to analyze the location, delimitation, epithelial changes, induction of folliculo-sebaceous structures, cellularity, vascularity, collagen pattern, and types of composite cells of the FH. In the majority of the biopsies, we confirmed the classical histopathologic features of fibrous histiocytomas. The presence of lymphoid nodules, mast cells, and sparse infiltrate of inflammatory cells was an interesting finding observed in our study.


Subject(s)
Humans , Histiocytoma, Benign Fibrous/pathology , Skin Neoplasms/pathology , Retrospective Studies
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