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Central Nervous System
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
This is usually a cystic and peripherally located tumor in children and young adults, characterized by a mixture of spindle-like cells and mono-nucleated or multi-nucleated giant cells. There is often intracellular lipid accumulation and the marked presence of reticulin fibers. It is normally a low-grade tumor. The major treatment modality is surgery, and complete excision is associated with the best outcome. However, higher-grade tumors are found, and an anaplastic variant is included in WHO 2016 classification. Radiation treatment may be of value in the management of more aggressive tumors or those that recur after surgery.63 Up to 60% of tumors may harbor BRAFV600E mutations.
Role of Autoimmunity in Gluten-Sensitive Enteropathy
Published in Tadeusz P. Chorzelski, Ernst H. Beutner, Vijay Kumar, Tadeusz K. Zalewski, Serologic Diagnosis of Celiac Disease, 2020
Ernst H. Beutner, Tadeusz P. Chorzelski, Vijay Kumar, J. Edward Valeski, Thomas Rossi
Some understanding of the pathogenic significance of IgA-EmA derives from the nature and distribution of the antigens with which they react. Morphologically, their structure is that of extracellular matrix components formed by smooth muscle cells. They lie on and between the myofibrils55 (see Chapter 7 for illustration). The most reactive forms in normal tissue occur in the smooth muscle fibers close to lymphoid tissue or epithelium of the gastrointestinal tract, spleen, lymph nodes, or thymus. The IgA-EmA antigen appears to be synthesized by smooth muscle myocytes and is apparently modulated by their proximity to lymphoid cells, as demonstrated by their organ specificity.55 That is, they give maximal reactivity with selected smooth muscles, lymphoid tissue, and spleen. The role of the proximity of the gastrointestinal tract epithelium or lymphoid tissue is well illustrated by the observations that while smooth muscles of arteries in distal areas are either very weakly reactive or essentially nonreactive with IgA-EmA, arteries in the skeletal muscle of the upper third of the esophagus (which has no smooth muscle layers) are strongly reactive (Figure 1). This example of the interrelationship between the arterial smooth muscle matrix components and surrounding cellular elements illustrates the long-recognized interrelationships of cells to reticulin.50 (Reticulin refers to the morphological structure of some insoluble matrix components.)
Peripheral Blood and Bone Marrow
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Fermina Maria Mazzella, Gerardo Perrotta
At the time of sign-out, a routine bone marrow “case” consists of a peripheral blood smear from the day of the biopsy, one to three Wright-Giemsa-stained bone marrow aspirate smears, one to three touch imprints, three levels of the bone marrow biopsy and bone marrow clot, a Prussian blue-stained bone marrow aspirate smear (iron stain), and a periodic acid Schiff (PAS)-stained bone marrow biopsy and clot section. In special situations, such as immunosuppressed or AIDS patients, stains for acid fast bacilli and fungi are performed automatically, as these patients are often incapable of forming the tell-tale granulomata. Also, silver stain for reticulin fibers is routinely performed, as in this patient population the reticular network is invariably increased.
Pathologic and Immunophenotypic Characterization of Syncytial Giant Cell Variant of Pediatric Hepatocellular Carcinoma. A Distinct Subtype
Published in Fetal and Pediatric Pathology, 2023
Mukul Vij, Jagadeesh Menon, Komalavalli Subbiah, Lexmi Priya Raju, Gowripriya Gowrisankar, Naresh Shanmugum, Ilankumaran Kaliamoorthy, Ashwin Rammohan, Mohamed Rela
The explanted liver showed greenish discoloration and had multiple distinct whitish nodules ranging in sizes from 0.1 cm to 1.2 cm (Fig. 4B). Segment 5 shows an encapsulated distinct white nodule corresponding to the neoplastic lesion reported on imaging. Microscopy demonstrated distorted lobular architecture with micronodular cirrhotic transformation (Fig. 4C). Macroregenerative nodules were noted. There was variable portal inflammation, prominent portal/periportal cholangiolar proliferation with ductular bile plugs, and lobular bilirubinostasis. Diffuse sinusoidal fibrosis was highlighted by the trichrome stain (Fig. 4D). A single tumor nodule measuring 6 mm with neoplastic cells arranged in sheets and displaying clear cytoplasm was identified (Fig. 4E). Diffuse syncytial giant cell transformation containing 4 to 25 nuclei was also noted. Cholestasis and hemopoiesis was observed. There was no vascular or perineural invasion. Reticulin was decreased.
Circulating YKL-40 in Philadelphia-negative myeloproliferative neoplasms
Published in Acta Clinica Belgica, 2021
Ivan Krečak, Velka Gverić-Krečak, Ivana Lapić, Pavle Rončević, Josipa Gulin, Ksenija Fumić, Filip Krečak, Hrvoje Holik, Nadira Duraković
BM fibrosis in MPNs is the result of complex and poorly understood interactions among megakaryocytes, fibroblasts, endothelial cells, inflammatory cytokines and marrow stroma. Although there is evidence suggesting that myelofibrosis osteoclasts and fibroblasts might be clonal and functionally impaired [38], the current dogma is that stromal changes are secondary to the cytokine release produced by the hematopoietic clone cells in MPN patients [13]. YKL-40 has been shown to be a growth factor for fibroblasts and several studies have demonstrated an important role of YKL-40 in extracellular matrix remodeling [18,22,23]. In our study, higher serum YKL-40 levels were associated with the presence of reticulin fibers in the BM of ET and PV patients; reticulin fibrosis has been cited as a risk factor for MF transformation [13]. However, we did not detect positive correlation between serum YKL-40 levels and BM fibrosis grades in MF patients. It was most likely due to the limited number of MF patients included and because our study sample was predominated with MF patients presenting with grade 3 BM fibrosis (Table 3). In this perspective, positive association of serum YKL-40 levels with reticulin fibrosis in ET and PV might implicate the potential role of circulating YKL-40 in promoting disease progression and BM fibrosis in MPNs through inflammation-induced tissue repair processes. However, additional studies are needed to confirm our speculations and elucidate the cell(s) of serum YKL-40 origin in MPNs.
TAFRO syndrome complicated with occlusion of multiple cerebral arteries
Published in Modern Rheumatology Case Reports, 2018
Takeshi Iwasaki, Yuri Nakamura, Shinnosuke Nakayama, Natsuko Koita, Takakuni Maki, Yoshiki Iemura, Hironori Haga, Tsutomu Okada, Shuji Akizuki, Nobuo Kuramoto, Kosaku Murakami, Ran Nakashima, Hajime Yoshifuji, Tsuneyo Mimori, Koichiro Ohmura
When we reviewed the disease course, this case meets the diagnostic criteria of TAFRO syndrome proposed by both Masaki et al. [8] and Iwaki et al. [6]. However, on admission only Iwaki’s diagnostic criteria was fulfilled. Since the sensitivity of Masaki’s criteria was only 64% (18/28) [8], Iwaki’s criteria might be better for diagnosis during the early course of the disease, although the sensitivity and specificity of the criteria were not described. On admission, our patient showed pathological findings of lymph nodes compatible with Castleman’s disease, anasarca, fever (before admission), hepatosplenomegaly, absence of hypergammaglobulinemia, small volume lymphadenopathy, normoplasia of megakaryocytes in bone marrow, and high levels of serum ALP, but absence of thrombocytopenia and renal insufficiency. Since bone marrow biopsy was not performed, presence of reticulin fibrosis of bone marrow was not clear. Before thrombocytopenia and renal insufficiency became evident, it was difficult to make a correct diagnosis; however, anasarca and high ALP without hypergammaglobulinemia were characteristics of TAFRO syndrome. Even though the fibrosis of bone marrow was not present, it is not inconsistent with TAFRO syndrome, because it was reported that about 20% of TAFRO syndrome’s cases showed no bone marrow fibrosis [6]. Leucocytosis on admission is probably due to TAFRO syndrome, because there were no signs of infection after intensive survey and no suspicious medication, and also because leukocytosis is often seen in TAFRO syndrome [8].