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Granulomatous Diseases
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Albert Alhatem, Robert A. Schwartz, Muriel W. Lambert, W. Clark Lambert
Laboratory studies: A complete blood count is essential. Biopsy of a skin lesion is a crucial part of the diagnosis, and it shows masses of lymphocytes in which collections of histiocytes are noted. The hallmark of the disease is emperipolesis or histiocytes with lymphocytes within their cytoplasm. The histiocytes stain positive for S-100 and CD68 and negative for CD1a and langerin.
Histiocytosis and Lipid Storage Diseases
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Salwa Shabbir Sheikh, David F. Garvin
b. Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease). Rosai-Dorfman disease (RDD) was first recognized in 1969 as an uncommon proliferative histiocytic disorder (Fig. 3). It is a benign, self-limiting disorder of unknown etiology. Some cases are associated with Epstein-Barr virus, herpes virus 6 infection, and lymphoma. It usually occurs in the first two decades of life. There is a predilection for children, especially <10 years, with the clinical picture of inflammation, i.e., fever, leukocytosis, high ESR, lymphadenopathy, and systemic symptoms. Significant numbers of cases appear in children with immunologic disorders, e.g., Wiskott-Aldrich syndrome, autoimmune hemolytic disease, and glomerulonephritis. The entity was originally described in lymph nodes, but has been reported to involve almost every organ system. Almost half of patients with RDD have at least one site of extra-nodal disease. In this hemophagocytic syndrome the internalized lymphocytes are intact, suggesting that they entered the histiocytes by emperipolesis rather than phagocytosis. Usually the course is benign and requires no therapy, but steroids and chemotherapy are used in severe cases.
Hematopoietic System
Published in Pritam S. Sahota, James A. Popp, Jerry F. Hardisty, Chirukandath Gopinath, Page R. Bouchard, Toxicologic Pathology, 2018
Kristin Henson, Tanasa Osborne, Gregory S. Travlos
Platelet production begins with the megakaryoblast, which is a large, single nucleated cell with deeply basophilic cytoplasm. The megakaryoblast undergoes endomitosis, resulting in a larger multinucleated cell with increased amounts of moderately basophilic cytoplasm. As maturation progresses, the megakaryocyte cytoplasm develops numerous eosinophilic granules. Emperipolesis, the movement of blood cells (neutrophils, erythrocytes, lymphocytes) within megakaryocytes, is fairly common with up to 5 percent of megakaryocytes containing blood cells in normal bone marrow from humans (Centurione et al. 2004; Harvey 2001). Emperipolesis differs from phagocytosis in that the blood cells exist temporarily within the megakaryocyte (Harvey 2001). Emperipolesis has been described as a random process, although other proposed explanations include facilitation of cell traffic across the marrow–blood barrier, particularly with a high demand for blood cells or as a sanctuary for normal granulocytes in an unfavorable marrow environment (reviewed in Tanaka et al. 1997). The cytologic appearance of erythroid, myeloid, and megakaryocytic cells, including examples of rodent ring forms, is found in Figures 15.3 and 15.4.
Megakaryocyte emperipolesis: a new frontier in cell-in-cell interaction
Published in Platelets, 2020
Pierre Cunin, Peter A. Nigrovic
The observations that emperipolesis is preserved across species and tightly regulated with environmental context strongly suggests that emperipolesis has a physiological purpose. An early speculation was that MKs provide a “sanctuary” for neutrophils in an unfavorable marrow environment, though it is not clear under which conditions such shelter should be necessary [43,57]. Others have suggested that emperipolesis arises because the space constraints of the marrow force cells into MKs through channels between the DMS and the cell surface [51]. Indeed, very limited data have suggested that some ‘emperipolesed’ cells may be in a compartment accessible to a membrane-impermeable tracer, indicating a topological location contiguous with the extracellular environment [58]. However, at least as a general explanation of emperipolesis, this hypothesis no longer appears compatible with the known cell biology [21]. A further suggestion is that emperipolesis could be a route for blood cells from the marrow to the circulation, in particular since erythrocytes and their precursors are sometimes observed within MKs [56]. Focusing in particular on MK emperipolesis of neutrophils, two teleological questions arise: why would an MK seek to internalize neutrophils, and why would a neutrophil seek to pass through an MK?
An update on the pharmacological management of autoimmune hepatitis
Published in Expert Opinion on Pharmacotherapy, 2021
Yooyun Chung, Mussarat N Rahim, Jonathon J Graham, Yoh Zen, Michael A Heneghan
Liver histology is crucial for the diagnosis of AIH. Hallmark features include interface hepatitis with plasma cell infiltration of the portal tracts, emperipolesis and hepatic rosette formation (Figure 1) [5]. Interface hepatitis denotes inflammation at the portal and parenchymal interface. Emperipolesis refers to the engulfment of lymphocytes by hepatocytes. Rosette formation is not always seen but represents regeneration of hepatocytes. The presence of all three features is convincing of AIH, whereas interface hepatitis and plasma cell infiltration without the other features are still considered compatible with a diagnosis of AIH according to the simplified diagnostic criteria. One-third of patients will have established cirrhosis at time of diagnosis [5].
Nasal septum lesions causing diagnostic difficulty: Case report
Published in Acta Oto-Laryngologica Case Reports, 2023
Aiko Kishino, Syuji Yonekura, Ichiro Fukumoto, Tomoyuki Arai, Tomohisa Iinuma, Takeshi Suzuki, Kazuki Yamasaki, Yuichiro Otsuka, Manayu Shiina, Masayuki Ota, Jun-ichiro Ikeda, Toyoyuki Hanazawa
The histopathologic examination showed marked histiocytic infiltration accompanied by mononuclear cells, such as lymphocytes and plasma cells. Emperipolesis, a process by which one cell engulfs and temporarily retains another cell within its cytoplasm, has been observed in immune cells, particularly macrophages (Figure 4(A)). In the present study, a small number of histiocytes demonstrated emperipolesis. An immunohistochemical analysis revealed positive staining for S-100 protein and CD68 but negative staining for CD1a (Figure 4(B–D)). These pathological findings supported the diagnosis of extranodal Rosai-Dorfman disease (RDD). At 19 months after surgery, marked improvement in nasal congestion was observed. No postoperative treatment was administered.