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1.
Int Ophthalmol ; 44(1): 236, 2024 Jun 20.
Artículo en Inglés | MEDLINE | ID: mdl-38902584

RESUMEN

PURPOSE: Histiocytosis is one of the most challenging diseases in medical practice. Because of the broad spectrum of clinical manifestations, systemic involvements, unknown etiology, and complex management, different types of histiocytosis are still a big question mark for us. Orbital histiocytosis is characterized by the abnormal proliferation of histiocytes in orbital tissues. It could affect the orbit, eyelid, conjunctiva, and uveal tract. Orbital histiocytosis can cause limited eye movement, proptosis, decreased visual acuity, and epiphora. In this study, we review the novel findings regarding the pathophysiology, diagnosis, and treatment of different types of histiocytosis, focusing on their orbital manifestations. METHOD: This review was performed based on a search of the PubMed, Scopus, and Embase databases or relevant published papers regarding orbital histiocytosis on October 9th, 2023. No time restriction was proposed, and articles were excluded if they were not referenced in English. RESULTS: 391 articles were screened, most of them being case reports. The pathophysiology of histiocytosis is still unclear. However, different mutations are found to be prevalent in most of the patients. The diagnostic path can be different based on various factors such as age, lesion site, type of histiocytosis, and the stage of the disease. Some modalities, such as corticosteroids and surgery, are used widely for treatment. On the other hand, based on some specific etiological factors for each type, alternative treatments have been proposed. CONCLUSION: Significant progress has been made in the detection of somatic molecular changes. Many case studies describe various disease patterns influencing the biological perspectives on different types of histiocytosis. It is necessary to continue investigating and clustering data from a broad range of patients with histiocytosis in children and adults to define the best ways to diagnose and treat these patients.


Asunto(s)
Histiocitosis , Enfermedades Orbitales , Humanos , Histiocitosis/diagnóstico , Enfermedades Orbitales/diagnóstico , Enfermedades Orbitales/etiología , Histiocitos/patología
2.
Blood Cancer J ; 14(1): 92, 2024 May 31.
Artículo en Inglés | MEDLINE | ID: mdl-38821935

RESUMEN

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare lymphoma with sparse tumor B-cells and a favorable prognosis. Variant growth patterns of NLPHL, however, often show advanced stage, progression to T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) and a worse prognosis. We studied the tumor microenvironment (TME) of NLPHL and THRLBCL using highplex imaging and spatial profiling at the single cell level. Our findings show distinct differences in TME composition and spatial configuration that differ among typical and variant NLPHL and THRLBCL. Typical NLPHL show abundant helper T-cell subsets, while THRLBCL show abundant cytotoxic T-cells and macrophages. Tumor B-cell size and content is lowest in typical NLPHL, followed by variant NLPHL, and highest in THRLBCL, whereas an opposite trend characterized TME B-cells. CD4/CD8 double-positive T-cells are seen in all NLPHL but not in the majority of THRLBCL and are spatially distant from LP-cells and TFH-rosettes. The differences in macrophage/monocyte content in distinguishing NLPHL pattern E from THRLBCL is further corroborated in independent cohorts of cases. Our results validate the current approach to classification and in addition provide novel insights that could be leveraged to refine clinical management for patients with this spectrum of lymphomas.


Asunto(s)
Enfermedad de Hodgkin , Linfoma de Células B Grandes Difuso , Microambiente Tumoral , Humanos , Enfermedad de Hodgkin/patología , Enfermedad de Hodgkin/diagnóstico , Linfoma de Células B Grandes Difuso/patología , Linfoma de Células B Grandes Difuso/diagnóstico , Masculino , Histiocitos/patología , Femenino , Persona de Mediana Edad , Adulto , Linfocitos T/patología , Linfocitos T/inmunología
3.
Acta Neuropathol Commun ; 12(1): 78, 2024 05 20.
Artículo en Inglés | MEDLINE | ID: mdl-38769536

RESUMEN

Neurologic Rosai-Dorfman disease (RDD) is a rare type of non-Langerhans cell histiocytosis that affects the central nervous system. Most neurologic RDDs grow like meningiomas, have clear boundaries, and can be completely resected. However, a few RDDs are invasive and aggressive, and no effective treatment options are available because the molecular mechanisms involved remain unknown. Here, we report a case of deadly and glucocorticoid-resistant neurologic RDD and explore its possible pathogenic mechanisms via single-cell RNA sequencing. First, we identified two distinct but evolutionarily related histiocyte subpopulations (the C1Q+ and SPP1+ histiocytes) that accumulated in the biopsy sample. The expression of genes in the KRAS signaling pathway was upregulated, indicating gain-of-function of KRAS mutations. The C1Q+ and SPP1+ histiocytes were highly differentiated and arrested in the G1 phase, excluding the idea that RDD is a lympho-histio-proliferative disorder. Second, although C1Q+ histiocytes were the primary RDD cell type, SPP1+ histiocytes highly expressed several severe inflammation-related and invasive factors, such as WNT5A, IL-6, and MMP12, suggesting that SPP1+ histiocytes plays a central role in driving the progression of this disease. Third, oligodendrocytes were found to be the prominent cell type that initiates RDD via MIF and may resist glucocorticoid treatment via the MDK and PTN signaling pathways. In summary, in this case, we report a rare presentation of neurologic RDD and provided new insight into the pathogenic mechanisms of progressive neurologic RDD. This study will also offer evidence for developing precision therapies targeting this complex disease.


Asunto(s)
Histiocitosis Sinusal , Análisis de la Célula Individual , Humanos , Masculino , Histiocitos/patología , Histiocitosis Sinusal/patología , Proteínas Proto-Oncogénicas p21(ras)/genética , Proteína Wnt-5a/metabolismo , Proteína Wnt-5a/genética , Persona de Mediana Edad
4.
Life Sci ; 348: 122692, 2024 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-38710283

RESUMEN

Erdheim Chester Disease (ECD) is a rare histiocytic disorder marked by infiltration of organs with CD68+ histiocytes. ECD stems from mutations of BRAF and MAP2K1 in hematopoietic stem and progenitor cells (HSPCs), which further differentiate into monocytes and histiocytes. Histopathology reveals lipid-containing histiocytes, which test positive for CD68 and CD133 in immunohistochemistry. Signs and symptoms vary and depend on the organ/s of manifestation. Definitive radiological results associated with ECD include hairy kidney, coated aorta, and cardiac pseudotumor. Treatment options primarily include anti-cytokine therapy and inhibitors of BRAF and MEK signaling.


Asunto(s)
Enfermedad de Erdheim-Chester , Enfermedad de Erdheim-Chester/tratamiento farmacológico , Enfermedad de Erdheim-Chester/diagnóstico , Enfermedad de Erdheim-Chester/genética , Enfermedad de Erdheim-Chester/terapia , Enfermedad de Erdheim-Chester/patología , Enfermedad de Erdheim-Chester/metabolismo , Humanos , Proteínas Proto-Oncogénicas B-raf/genética , Proteínas Proto-Oncogénicas B-raf/antagonistas & inhibidores , Histiocitos/patología , Histiocitos/metabolismo
5.
J Cutan Pathol ; 51(9): 658-661, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38769706

RESUMEN

Cytophagic histiocytic panniculitis (CHP) is associated with a number of systemic conditions and is characterized by the presence of benign phagocytic histiocytes ("bean bag cells"), including phagocytosed erythrocytes, leukocytes, and platelets. We describe a case of a 72-year-old female who presented with a papular eruption that clinically mimicked pityriasis lichenoides et varioliformis acuta (PLEVA). Given that her skin biopsy had multiple features concerning PLEVA, this diagnosis was classified as a superficial pityriasis lichenoides-like variant of CHP. The histopathologic presence of cytophagic histiocytosis prompted workup for a systemic malignancy, leading to a diagnosis of underlying acute monocytic leukemia of myeloid lineage.


Asunto(s)
Paniculitis , Humanos , Femenino , Anciano , Paniculitis/patología , Paniculitis/diagnóstico , Histiocitos/patología , Leucemia Mieloide Aguda/patología , Leucemia Mieloide Aguda/diagnóstico , Leucemia Monocítica Aguda/patología , Leucemia Monocítica Aguda/diagnóstico , Diagnóstico Diferencial , Diferenciación Celular , Monocitos/patología
6.
J Cutan Pathol ; 51(7): 506-512, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38606952

RESUMEN

Crystal-storing histiocytosis (CSH) is a rare condition in which crystals accumulate in the cytoplasm of histiocytes and is usually associated with a lymphoplasmacytic neoplasm. Cutaneous CSH is extraordinarily rare and limited to case reports in the literature. We report two cases of this disease with cutaneous involvement. Case 1 was a 65-year-old male with a 4-month history of a pruritic eruption that started as a solitary pink to skin-colored indurated plaque on the anterior neck before progressing to involve the whole neck, chest wall, and face. Case 2 was a 54-year-old woman with a history of unspecified "lymphoma" who presented with a soft nodule on the forearm. Biopsies from both cases had similar findings and showed a proliferation of epithelioid cells with pink cytoplasm and intracellular crystalline structures infiltrating the dermis and subcutaneous fat. In the first case, the cells were positive for CD43, CD45, CD68, and IgG kappa, and in the second case, the crystals were positive for IgG lambda. Based on these findings, the patients were diagnosed with cutaneous CSH. We highlight this rare diagnosis and the importance of investigating an underlying lymphoplasmacytic neoplasm.


Asunto(s)
Histiocitosis , Humanos , Anciano , Masculino , Femenino , Histiocitosis/patología , Histiocitosis/metabolismo , Persona de Mediana Edad , Histiocitos/patología , Histiocitos/metabolismo , Cristalización , Enfermedades de la Piel/patología , Enfermedades de la Piel/metabolismo
7.
Zhonghua Bing Li Xue Za Zhi ; 53(4): 364-369, 2024 Apr 08.
Artículo en Chino | MEDLINE | ID: mdl-38556820

RESUMEN

Objective: To investigate the clinicopathological features of Erdheim-Chester disease (ECD) initially diagnosed at extraskeletal locations. Methods: Clinical and pathological data of four cases of ECD diagnosed initially in extraskeletal locations were collected at Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, from January 2013 to June 2023. BRAF V600E gene was detected by reverse transcription polymerase chain reaction (RT-PCR). Pertinent literatures were reviewed. Results: Four ECD patients included two males and two females ranging in ages from 2 years 11 months to 69 years. The lesions located in the lung (two cases), central nervous system (one case), and the testicle (one case) were collected in the study. One patient had occasional fever at night, one had nausea and vomiting, and two were asymptomatic. Radiologically, the two pulmonary ECD showed diffuse ground-glass nodules in both lungs, and the lesions in central nervous system and testicle both showed solid masses. Microscopically, there were infiltration of foamy histiocyte-like cells and multinucleated giant cells in a fibrotic background, accompanied by varying amounts of lymphocytes and plasma cells. The infiltration of tumor cells in pulmonary ECD was mainly seen in the subpleural area, interlobular septa, and perivascular and peribronchiolar areas. The fibrosis was more pronounced in the pleura and interlobular septa, and less pronounced in the alveolar septa. Immunohistochemical staining showed that all tumor cells expressed CD68, CD163 and Fô€ƒ¼a; one case showed S-100 expression; three cases were positive for BRAF V600E; all were negative for CD1α and Langerin. RT-PCR in all four cases showed BRAF V600E gene mutation. Conclusions: Extraskeletal ECD is often rare and occult, and could be easily misdiagnosed, requiring biopsy confirmation. The radiologic findings of pulmonary ECD is significantly different from other types of ECD, and the histopathological features of pronounced infiltration in the subpleura area, interlobular septa, perivascular and peribronchiolar areas can be helpful in the differential diagnosis from other pulmonary diseases. Detection of BRAF V600E gene mutation by RT-PCR and its expression by immunohistochemical staining are also helpful in the diagnosis.


Asunto(s)
Enfermedad de Erdheim-Chester , Masculino , Femenino , Humanos , Enfermedad de Erdheim-Chester/patología , Proteínas Proto-Oncogénicas B-raf/genética , Pulmón/patología , Histiocitos/patología , Sistema Nervioso Central/patología , Mutación
8.
J Med Case Rep ; 18(1): 182, 2024 Mar 27.
Artículo en Inglés | MEDLINE | ID: mdl-38532442

RESUMEN

BACKGROUND: Rosai-Dorfman disease (RDD) is a form of non-Langerhans cell histiocytosis in which the activated histiocytes of the lymph nodes and other organs begin to accumulate following excessive production. Bilateral, massive, and painless lymphadenopathy are classic presentations. Systemic RDD is already known to be a rare condition, but isolated cutaneous RDD is extremely rare. We presented a rare and unusual presentations of a disease. CASE PRESENTATION: A 35-year-old Thai female with a 6-month history of a small acne-like lesion that rapidly progressed to 5 cm tumor-like lesions on the face within 3 months. Tissue histology showed a dense dermal infiltration of histiocytes with emperipolesis phenomenon. Immunohistochemistry was positive for S100 protein and CD68 and negative for CD1a. Oral prednisolone (50 mg/day) was initiated with a favorable outcome at the one-month follow-up. However, prednisolone yielded a partial response at 2-month follow-up, leading to application of another modality. CONCLUSION: Although cutaneous Rosai-Dorfman disease is considered benign and well medical responded disease, patients with atypical presentation and rapid growing lesion may necessitate aggressive multimodal treatment.


Asunto(s)
Histiocitosis Sinusal , Linfadenopatía , Enfermedades de la Piel , Humanos , Femenino , Adulto , Histiocitosis Sinusal/metabolismo , Histiocitosis Sinusal/patología , Enfermedades de la Piel/patología , Histiocitos/metabolismo , Histiocitos/patología , Linfadenopatía/patología , Prednisolona
9.
Indian J Pathol Microbiol ; 67(1): 185-188, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38358218

RESUMEN

Juvenile xanthogranuloma is a benign self-limiting lesion commonly described in infants and young children. It most commonly involves the skin presenting as single or multiple yellowish-brown papules. Clinical scenario with the classic histomorphology showing histiocytic aggregates in the dermis with xanthomatous cytoplasm, toutan type giant cells, immunohistochemistry with positive CD68, CD163, factor XIIIa and negative CD1a and S-100 help in diagnosis. However, diagnosis becomes challenging with predominant systemic bone marrow involvement in post-B-lymphoblastic leukemia settings.


Asunto(s)
Leucemia-Linfoma Linfoblástico de Células Precursoras B , Leucemia-Linfoma Linfoblástico de Células Precursoras , Xantogranuloma Juvenil , Xantomatosis , Lactante , Niño , Humanos , Preescolar , Médula Ósea/patología , Piel/patología , Xantogranuloma Juvenil/diagnóstico , Xantogranuloma Juvenil/patología , Histiocitos/patología , Xantomatosis/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras B/patología , Leucemia-Linfoma Linfoblástico de Células Precursoras/patología
10.
Cardiovasc Pathol ; 70: 107625, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38417471

RESUMEN

Erdheim-Chester disease (ECD) is a rare histiocytosis characterized by the foamy CD68+CD1a- histiocytes infiltrating multiple organs and tissues. ECD might be asymptomatic or present with variable manifestations. The diagnosis of ECD requires characteristic radiological findings and pathological features. Herein, we described a 52-year-old female patient who was admitted to our hospital for recurrent pericardial effusion for two months. She has a medical history of papillary thyroid carcinoma (PTC) and underwent a total thyroidectomy two years before admission. The radiological findings suggested a potential diagnosis of ECD. Cytological analysis of the effusion cytology specimen revealed CD68+CD1a- histiocytes, confirming the ECD diagnosis. The BRAF V600E mutation was identified in the histiocytes, prompting the administration of vemurafenib, a BRAF inhibitor. After two months of standard-dose vemurafenib treatment, the disease was well controlled with pericardial effusion regression.


Asunto(s)
Enfermedad de Erdheim-Chester , Derrame Pericárdico , Proteínas Proto-Oncogénicas B-raf , Vemurafenib , Humanos , Enfermedad de Erdheim-Chester/genética , Enfermedad de Erdheim-Chester/tratamiento farmacológico , Enfermedad de Erdheim-Chester/complicaciones , Enfermedad de Erdheim-Chester/patología , Enfermedad de Erdheim-Chester/diagnóstico , Femenino , Derrame Pericárdico/patología , Derrame Pericárdico/etiología , Persona de Mediana Edad , Proteínas Proto-Oncogénicas B-raf/genética , Vemurafenib/uso terapéutico , Resultado del Tratamiento , Mutación , Histiocitos/patología , Inhibidores de Proteínas Quinasas/uso terapéutico , Valor Predictivo de las Pruebas , Citología
11.
Indian J Pathol Microbiol ; 67(3): 641-644, 2024 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-38391345

RESUMEN

ABSTRACT: Histiocytic disorders mostly occur as de-novo nodal or extranodal benign masses with rare secondary malignant transformation. A 10-year-old female presented with 10-cm cervical swelling since 9 months associated with fever. Computed tomography revealed left cervical lymphadenopathy and bilateral lung nodules. Lymph node excision biopsy showed effacement of architecture by atypical histiocytes with marked nuclear pleomorphism and frequent mitosis. Focal areas showed mature histiocytes with emperipolesis. The cells were immunopositive for CD68, CD163, and S100 (focal), whereas they were negative for Langerin and CD1a. The Ki67 proliferative index was 30%. A diagnosis of histiocytic sarcoma in a background of Rosai-Dorfman disease was made.


Asunto(s)
Antígenos CD , Antígenos de Diferenciación Mielomonocítica , Sarcoma Histiocítico , Histiocitosis Sinusal , Inmunohistoquímica , Tomografía Computarizada por Rayos X , Humanos , Femenino , Sarcoma Histiocítico/diagnóstico , Sarcoma Histiocítico/patología , Histiocitosis Sinusal/diagnóstico , Histiocitosis Sinusal/patología , Niño , Antígenos CD/análisis , Proteínas S100/análisis , Histiocitos/patología , Ganglios Linfáticos/patología , Biopsia , Histocitoquímica , Transformación Celular Neoplásica , Microscopía , Receptores de Superficie Celular/genética , Linfadenopatía/patología , Molécula CD68
12.
Vet Clin Pathol ; 53(1): 122-130, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38242688

RESUMEN

A 7-month-old intact female bearded collie dog was admitted after a 2-week history of progressive cough, inappetence, and lethargy, with no response to previous treatment with doxycycline and steroids. Mild attenuation of lung sounds in the right middle hemithorax was the only abnormality detected on physical examination. Abdominal ultrasound and thoracic radiographs were performed and revealed multifocally distributed nodules and masses, well-circumscribed and of variable size in the kidneys and pulmonary parenchyma. Ultrasound-guided fine needle aspirates of the renal and pulmonary masses were taken. A cytologic evaluation of these lesions pointed towards a malignant mesenchymal neoplasia. Euthanasia was elected due to the poor prognosis and rapid progression. The post-mortem histopathology, a positive result to IBA1 immunoperoxidase staining, and a lack of detection of infectious agents, and negative E-cadherin immunostaining enabled the final diagnosis of a disseminated histiocytic sarcoma. We report an atypical form, both in breed and age, of canine disseminated histiocytic sarcoma. While all breeds can be affected, there is a clear predisposition in some, and no cases have been previously described in bearded collies. Moreover, to the authors' knowledge, this is the youngest dog with this histiocytic disorder described to date. Disseminated histiocytic sarcoma should be considered as a differential diagnosis of multinodular tumors in dogs, regardless of the anatomic location and age of the dogs, even in puppies.


Asunto(s)
Enfermedades de los Perros , Sarcoma Histiocítico , Sarcoma , Perros , Animales , Femenino , Sarcoma Histiocítico/patología , Sarcoma Histiocítico/veterinaria , Sarcoma/patología , Sarcoma/veterinaria , Biopsia con Aguja Fina/veterinaria , Histiocitos/patología , Enfermedades de los Perros/diagnóstico
13.
Histopathology ; 84(5): 837-846, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38213281

RESUMEN

AIMS: The discovery of somatic genetic alterations established many histiocytic disorders as haematologic neoplasms. We aimed to investigate the demographic characteristics and additional haematologic cancers of patients diagnosed with histiocytic disorders in The Netherlands. METHODS AND RESULTS: We retrieved data on histiocytosis patients from the Dutch Nationwide Pathology Databank (Palga). During 1993 to 2022, more than 4000 patients with a pathologist-assigned diagnosis of a histiocytic disorder were registered in Palga. Xanthogranulomas were the most common subtype, challenging the prevailing assumption that Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder. LCH and juvenile xanthogranuloma (JXG) had a peak incidence in the first years of life; males were overrepresented among all histiocytosis subgroups. 118 patients had a histiocytic disorder and an additional haematologic malignancy, including 107 (91%) adults at the time of histiocytosis diagnosis. In 16/118 patients, both entities had been analysed for the same genetic alteration(s). In 11 of these 16 patients, identical genetic alterations had been detected in both haematologic neoplasms. This included two patients with PAX5 p.P80R mutated B cell acute lymphoblastic leukaemia and secondary histiocytic sarcoma, further supporting that PAX5 alterations may predispose (precursor) B cells to differentiate into the myeloid lineage. All 4/11 patients with myeloid neoplasms as their additional haematologic malignancy had shared N/KRAS mutations. CONCLUSIONS: This population-based study highlights the frequency of xanthogranulomas. Furthermore, our data add to the growing evidence supporting clonal relationships between histiocytic/dendritic cell neoplasms and additional myeloid or lymphoid malignancies. Particularly adult histiocytosis patients should be carefully evaluated for the development of these associated haematologic cancers.


Asunto(s)
Neoplasias Hematológicas , Histiocitosis de Células de Langerhans , Adulto , Masculino , Humanos , Histiocitosis de Células de Langerhans/epidemiología , Histiocitosis de Células de Langerhans/genética , Histiocitosis de Células de Langerhans/patología , Histiocitos/patología , Neoplasias Hematológicas/genética , Neoplasias Hematológicas/patología , Células Dendríticas/patología , Demografía
14.
Am J Surg Pathol ; 48(4): 426-436, 2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-37988030

RESUMEN

Tissue eosinophilia is seldom reported in B-cell lymphoma. It poses diagnostic challenges and frequently leads to the consideration of other diagnoses, particularly T-cell lymphomas. The scarce literature underscores the need for in-depth studies to enhance awareness and understanding of this phenomenon. We investigated 54 cases of B-cell lymphoma with notable tissue eosinophils, analyzing clinical information, hematoxylin and eosin staining, immunohistochemistry, and PCR-based clonality analysis. Nodal marginal zone lymphoma (NMZL) emerged as the most prevalent type (n=26), followed by B-cell lymphoma, not otherwise specified (n=13), diffuse large B-cell lymphoma (n=10), follicular lymphoma (n=2), chronic lymphocytic leukemia/small lymphocytic lymphoma (n=1), extranodal marginal zone lymphoma (n=1), and primary cutaneous marginal zone lymphoma (n=1). Shared features across different lymphoma types, best exemplified by NMZL, included plasmacytic differentiation (57.7%), increased vascularity (84.6%) with a tendency for perivascular distribution of neoplastic cells, and a tumor microenvironment abundant in T cells and histiocytes; some cases showed increased PD-1-positive cells. These features often raise consideration of angioimmunoblastic T-cell lymphoma. Along with clonality analysis, features supporting the diagnosis of B-cell lymphoma included cytological atypia in B cells rather than T cells, and the lack of follicular dendritic cell meshwork expansion. In addition, diffuse large B-cell lymphoma frequently exhibited interfollicular distribution and monocytoid appearance, indicating the possibility of transformed NMZL. Collectively, tissue eosinophilia can occur in diverse B-cell lymphomas but is most prevalent in tumors with a postgerminal stage of differentiation.


Asunto(s)
Eosinofilia , Linfoma de Células B de la Zona Marginal , Linfoma de Células B Grandes Difuso , Humanos , Eosinófilos/patología , Histiocitos/patología , Linfoma de Células B de la Zona Marginal/patología , Linfoma de Células B Grandes Difuso/genética , Microambiente Tumoral
15.
Am J Dermatopathol ; 46(2): 121-125, 2024 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-38055958

RESUMEN

ABSTRACT: Immunohistochemically, histiocytosis differentiating into Langerhans cells is typically characterized by the expression of CD1a, S100, and varying degrees of Langerin. However, CD1a-positive but S100-negative histiocytosis is extremely rare in clinical practice. We present a case of a 9-year-old boy with multiple erythematous to brown dome-shaped nodules. Histopathologic examination revealed dermal infiltrates of histiocytic cells, exhibiting a distinctive immunohistochemical profile of CD68+, S100-, CD1a+, and Langerin-. This exceptional case may contribute to our understanding of the etiology and differentiation processes of histiocytic proliferative disorders.


Asunto(s)
Histiocitosis de Células de Langerhans , Masculino , Humanos , Niño , Histiocitosis de Células de Langerhans/diagnóstico , Inmunohistoquímica , Células de Langerhans/patología , Histiocitos/patología , Eritema/patología
16.
Int J Hematol ; 119(1): 93-98, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37989992

RESUMEN

Juvenile xanthogranuloma (JXG) is usually identified by Touton giant cells, so their absence can complicate diagnosis. We encountered a case of non-typical neonatal JXG lacking Touton giant cells, which was difficult to differentiate from aleukemic leukemia cutis because of overlapping histopathological characteristics. A 1 month-old girl presented with a blueberry muffin rash and multiple 1-2 cm nodules within the subcutaneous and deeper soft tissues. Blood tests revealed pancytopenia. The initial nodule biopsy showed mononuclear cell infiltration, suggestive of mature monocytes or histiocytes, but no Touton giant cells. Bone marrow examination showed no evidence of leukemia. Despite worsening of the rash, pancytopenia, and weight gain over the following month, the results of the second biopsy remained consistent with the initial findings. Consequently, we provisionally diagnosed aleukemic leukemia cutis and initiated chemotherapy. After two courses of chemotherapy, the pancytopenia improved, but the nodules only partially regressed. A third biopsy of the nodule was performed to evaluate the histological response, and revealed Touton giant cells, confirming the diagnosis of JXG. In conclusion, distinguishing non-typical JXG from aleukemic leukemia cutis is challenging. This case highlights the importance of multiple biopsies and the potential for histopathological maturation.


Asunto(s)
Exantema , Leucemia , Pancitopenia , Neoplasias Cutáneas , Xantogranuloma Juvenil , Femenino , Humanos , Lactante , Exantema/patología , Histiocitos/patología , Leucemia/patología , Pancitopenia/patología , Neoplasias Cutáneas/patología , Xantogranuloma Juvenil/diagnóstico , Xantogranuloma Juvenil/complicaciones , Xantogranuloma Juvenil/patología
17.
Vet Clin Pathol ; 52(4): 691-697, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37914537

RESUMEN

Feline pulmonary Langerhans cell histiocytosis (FPLCH) is a rare histiocytic proliferative disease of middle-aged to older domestic cats. Langerhans cells in the terminal airways proliferate and infiltrate the interstitium and the airways to a lesser degree, widely effacing normal parenchyma. Historically, definitive diagnosis has required postmortem evaluation where pulmonary lesions have a classic gross and histologic morphology. Here, we present the first documented antemortem diagnosis of FPLCH using bronchoalveolar lavage (BAL) cytology and immunocytochemistry (ICC) in a 9-year-old British shorthair mix. The cat had a 3-month history of respiratory difficulty that was refractory to steroids and antimicrobials. Pulmonary radiographs had marked diffuse changes with a complex bronchointerstitial and micronodular pattern. BAL cytology revealed neutrophilic inflammation and markedly increased histiocytes with morphology distinct from typical pulmonary macrophages. ICC characterized histiocytes as CD1a+ /E-cadherin+ /CD11b- /PanCK- , consistent with a Langerhans cell phenotype. The cat was humanely euthanized due to poor prognosis and presented for necropsy. Gross, histopathologic, immunophenotypic, and ultrastructural findings confirmed a diagnosis of FPLCH. Proliferative cells were E-cadherin+ /Iba-1+ /CD18+ /CD1a+ /CD5+ /MHCII+ /CD204- /CD4- ; transmission electron microscopy identified the presence of Birbeck granules in the proliferating histiocytes, consistent with previous reports of FPLCH.


Asunto(s)
Enfermedades de los Gatos , Neoplasias Hematológicas , Histiocitosis de Células de Langerhans , Gatos , Animales , Histiocitosis de Células de Langerhans/diagnóstico , Histiocitosis de Células de Langerhans/veterinaria , Histiocitosis de Células de Langerhans/patología , Histiocitos/patología , Histiocitos/ultraestructura , Pulmón/patología , Inmunohistoquímica , Neoplasias Hematológicas/patología , Neoplasias Hematológicas/veterinaria , Cadherinas , Enfermedades de los Gatos/diagnóstico , Enfermedades de los Gatos/patología
18.
19.
J Cardiothorac Surg ; 18(1): 284, 2023 Oct 10.
Artículo en Inglés | MEDLINE | ID: mdl-37817188

RESUMEN

Rosai-Dorfman disease (RDD) is currently considered a group of neoplastic diseases of unknown etiology, with monoclonal proliferation of histiocytes, showing unique histopathologic features and varying clinical presentation. Primary thymic RDD is an extremely rare extranodal form of this disorder. In this study, we describe the case of an otherwise healthy 64-year-old Chinese man who presented with an isolated, asymptomatic soft tissue density lesion in the anterior mediastinum detected by computed tomography. Histology of the surgical specimen revealed infiltration of thymic tissue by sheets of large histiocytes with mixed lymphocytes and plasma cells, and background fibrosis. Immunohistochemical staining of the histiocytes was positive for S100, CD68, CD163, OCT2 and cyclin D1, but negative for CD1a and BrafV600E expression, thus supporting a diagnosis of RDD. Primary thymic RDD is extremely rare and may be a diagnostic challenge when presenting as mediastinal lesion.


Asunto(s)
Histiocitosis Sinusal , Enfermedades Musculoesqueléticas , Masculino , Humanos , Persona de Mediana Edad , Histiocitosis Sinusal/diagnóstico , Histiocitosis Sinusal/cirugía , Histiocitosis Sinusal/patología , Histiocitos/metabolismo , Histiocitos/patología , Timo , Linfocitos/patología , Mediastino/patología
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