mast cell leukemia pathology outlines

Extranodal vs. Nodal vs. Splenic Marginal Zone Lymphomas . Learn more about the change. Yoo D, Lessin LS, Jensen WN. Approximately 20% of patients with systemic mastocytosis (SM) have an associated haematological, clonal, non-mast cell lineage disease, and most exhibit an associated myelogenous neoplasm. H Introduction. Contributed by A.M. Medina and coworkers. Reported herein is an autopsy case of mast cell leukemia, a rare form of systemic mastocytosis, complicated with portal hypertension. Sperr W Leukemia cutis (LC) refers to the specific infiltration of the skin by neoplastic leukemic cells, most often in conjunction with systemic leukemia.1,2 There is debate about the appropriate diagnostic terminology for LC, and various authors have used historic, related, and/or overlapping terms, including chloroma, extramedullary myeloid tumor, granulocytic sarcoma, and myeloid sarcoma. The differential diagnoses of indolent SM include well-differentiated SM, isolated bone marrow mastocytosis, smoldering SM, mast cell hyperplasia, monoclonal mast cell activation syndrome (which is an ill-defined state not fulfilling the criteria for SM), lymphoplasmacytic lymphoma (especially in cases with pronounced bone marrow lymphocytosis), and the so-called fibromastocytic lesion (which is also ill-defined, showing localized bone marrow fibrosis with increased numbers of spindle-shaped mast cells, but CD25 and KITD816V are missing). S The CBC revealed slight leukocytosis (WBC count, 9,100/μL [9.1 × 109/L]) with marked eosinophilia (eosinophil proportion, 40% [0.40]). Diffuse TROCI-bm is encountered in myelomastocytic leukemia, MCL, or chronic basophilic leukemia, and focal TROCI-bm is seen in the common type of SM, well-differentiated mastocytosis, and chronic basophilic leukemia, especially in accelerated phase chronic myeloid leukemia. C In all cases, judicious use of immunohistochemical staining is of paramount importance. Mast cell leukemia (MCL) is a very rare form of systemic mastocytosis (SM) and accounts for less than 0.5% of all mastocytosis. Jordan Feger It is crucial to examine blood and bone marrow smears to be able to diagnose MCL because the histologic picture in aggressive SM is indistinguishable from MCL in many cases. Mastocytosis is a morphologic diagnosis. The routine workup in suspected cases of SM includes the following: (1) bone marrow trephine biopsy specimen (>2 cm), (2) bone marrow smears, and (3) blood smears. Enlargement of the spleen and low red blood cells (anemia) may also occur. Splenic mastocytosis is rare.35,36 Similar to cases exhibiting “intestinal” mastocytosis, meticulous analysis of bone marrow biopsy specimens should reveal some compact infiltrates, thus enabling establishment of a diagnosis of SM with prominent infiltration of the spleen. Mastocytosis is a constellation of disorders where there are excessive proliferation and accumulation of pathologic mast cells in the … Figure S2 Autopsy findings of the liver, Atypical mast cells (CD2+, CD25+, CD117+) with toluidine blue metachromasia were found in the peripheral blood and on bone marrow aspiration smears. Mast cell leukemia is a rare and aggressive form of mastocytosis characterized by > 20% mast cells found in the bone marrow aspirates of patients with signs of systemic mastocytosis-related organ damage.The prognosis for patients with mast cell leukemia … B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) are the most common mature B-cell neoplasms and considered to be different manifestations of the same disease. Regarding the presence of KITD816V in blood leukocytes, this finding indicates that KITD816V is not restricted to mast cells (as in indolent SM) but can be found also in non–mast cell hematopoietic cells, allowing the diagnosis of smoldering SM manifesting with B findings (organomegaly) but lacking C findings. A 52‐year‐old woman presented with urticaria‐like skin symptoms, anemia, and thrombocytopenia. Outline. However, criteria for the diagnosis of SM, in particular SM-AHNMD, are usually not fulfilled because compact mast cell infiltrates are almost always missed and the KITD816V mutation is not detected. In MCL, patients experience massive and devastating proliferation of … Horny H-P J Fumo American Journal of Clinical Pathology, Volume 132, Issue 1, July 2009, ... mast cell sarcoma, and high-grade plasma cell neoplasms. Sperr All other causes excluded. Detection of mutated Kit (D816V) in the skin should prompt the suspicion of SM, and examination of a bone marrow biopsy specimen is strongly recommended in every such case. WR The case involved a 51-year-old man with “dermatitis,” mucosal ulcerations, and splenomegaly. Escribano Department of Pathology, Saitama Medical University, Iruma, Japan. Introduction. Li Metcalfe Myelomastocytic leukemia versus mast cell leukemia versus systemic mastocytosis associated with acute myeloid leukemia: a diagnostic challenge. Brockman W Sperr Contributed by I. Maric and coworkers. H-P Mantle Cell lymphoma is typically an aggressive, rare, form of non-Hodgkin lymphoma (NHL) that arises from cells originating in the “mantle zone.” MCL accounts for roughly six percent of all NHL cases in … 2.2. Increased bone-marrow mast-cell content and lymphoproliferative disorders have been previously linked. MCL is a fatal disease that almost always behaves aggressively, … Search for more papers by this author. The degree of bone marrow infiltration in this case amounts to up to 30% of the section area and, therefore, exceeds that of typical indolent SM or isolated bone marrow mastocytosis by far. Figure S1 Microscopic and immunohistochemical Cut sections revealed a mottled pink-red parenchyma. Zheng Y(1), Nong L(2), Liang L(1), Wang W(1), Li T(1). (Case 199) A, Bone marrow biopsy specimen showing significant eosinophilia with mature eosinophils and their precursors. Our objective was to employ a panel of cytochemical and immunohistochemical markers to determine which ones would be most useful in separating these two entities. Metcalfe Mast cell sarcoma is a rare tumor that may present in many different anatomic locations and age groups, and prognosis is generally poor. (Case 55) A, Dense mast cell infiltrate in colonic lamina propria (H&E). Mastocytosis is a morphologic diagnosis and should not be diagnosed on the basis of clinical findings alone. Cytoplasm usu. In all such cases, the meticulous study of the bone marrow reveals systemic disease, although the degree of tissue infiltration may vary widely. It may appear de novo or secondary to previous mastocytosis and shares more clinicopathologic aspects with systemic mastocytosis than with acute myeloid leukemia. Sotlar After chemotherapy and disappearance of the associated neoplasm, typical compact mast cell infiltrates were disclosed.14 As a rule, appropriate examination of the primary trephine biopsy specimen enables diagnosis of mastocytosis retrospectively, usually based on 3 minor criteria (spindling, CD25 expression, and KITD816V). CD68. Mastocytosis comprises a heterogeneous group of disorders characterized by expansion and accumulation of neoplastic mast cells (MCs) in 1 or more organ systems. The case involved a 67-year-old woman with colonic adenocarcinoma and a persistently elevated serum tryptase above 20 ng/mL. Wehrmann Mast cell leukemia is an extremely aggressive subtype of acute myeloid leukemia that usually occurs de novo but can, rarely, evolve from transformation of chronic myeloid leukemia into the more aggressive … et al. Author information: (1)Department of Pathology, Second Department of Internal Medicine, First Department of Pharmacology, Tohoku University, Sendai, Japan. P Atypical mast cells (CD2+, CD25+, CD117+) with toluidine blue metachromasia were found in the peripheral blood and on bone marrow aspiration smears. Differential Diagnosis of Acute Scrotum in Childhood and Adolescence with High-Resolution Duplex Sonography. ... Reported herein is an autopsy case of mast cell leukemia… G A et al. –Mast cell leukemia •Mast cell sarcoma •Extracutaneous mastocytoma acute chronic P Valent et al. SB Authors Angela R Arredondo 1 , Jason Gotlib, … Moreover, it is possible to exclude or assess an associated non–mast cell hematologic neoplasm that might be obscured in the spleen by mast cell infiltrates. Therefore, particularly in view of its rarity and often unusual appearances and the mostly low or minimal degree of tissue infiltration, pathologists should be familiar with the diagnostic criteria defined for mastocytosis not only to be able to assess or exclude true mastocytosis but also to recognize its mimickers.1,2 Mimickers of mastocytosis are reactive states of mast cell hyperplasia on the one hand and rare neoplastic hematologic disorders such as tryptase-positive acute myeloid leukemia (AML) or myelomastocytic leukemia on the other hand. Akin WR Sperr et al. The term leukemia cutis is perhaps the most broad and is frequently used in the dermatopathology literature. JH Department of Molecular Pathology and Tumor Pathology and. K Microscopic. The minor diagnostic criteria are as follows: (1) prominent spindling of mast cells (>25%), (2) atypical immunophenotype of mast cells with expression of CD25, (3) activating point mutation of c-kit in codon 816 (usually KITD816V), and (4) chronically elevated serum tryptase level (>20 ng/mL). A RP Cytogenetic. are hemorrhagic. It furthers the University's objective of excellence in research, scholarship, and education by publishing worldwide, This PDF is available to Subscribers Only. General. F MAST CELL SARCOMA 1, 2. Simonitsch H F (c–d) Tumor Scattered spindle-shaped mast cells are hardly detectable (H&E). WR Horny Ghannadan Guenther Mast cell sarcoma is often misdiagnosed because the presenting cells bear little resemblance to normal mast cells and spindle-shaped mast cells frequently seen in systemic mastocytosis. Although histologically mast cell infiltration amounted to 20% to 30% of the section area, the number of atypical mast cells in the smear preparation was low and did not exceed 4% of all nucleated bone marrow cells. SR Please note: Wiley‐Blackwell are not responsible for the content or functionality of any supporting materials supplied by the authors. To establish a diagnosis of aggressive SM, signs of organ dysfunction (C findings) should be present. Occult mastocytosis is used as a preliminary and descriptive term for rare cases of mastocytosis that were initially obscured by a malignant hematologic disorder in the setting of SM-AHNMD. If you do not receive an email within 10 minutes, your email address may not be registered, L K K Bone-marrow mast cells in lymphoproliferative disorders. et al. Leriche If adequate histologic analysis of bone marrow is performed in cases with prominent mucosal infiltration, all turn out to belong to the SM subcategory. This article is an introduction to lymphoma.An introduction to lymph nodes and lymph node pathology that is not lymphoma are in the articles lymph node and lymph node pathology… Surgical Pathology … Author information: (1)Department of Pathology, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China. et al. Chronic basophilic leukemia is a very rare myeloproliferative neoplasm usually manifesting as secondary “basophilic crisis” in preexisting chronic myeloid leukemia. Travis Expression of CD25 on mast cells is defined as a minor diagnostic criterion and is usually seen only in mastocytosis but not in reactive states of mast cell hyperplasia. This is a very typical case of MPNEo exhibiting an increase in atypical mast cells.28,38 However, the criteria for diagnosis of SM (or better, SM-AHNMD) were not fulfilled. Working off-campus? et al. Any queries (other than missing material) should be directed to the corresponding author for the article. There was also a mild normochromic anemia (hemoglobin level, 13.9 g/dL [139 g/L]) and thrombocytosis (platelet count, 605 × 103/μL [605 × 109/L]). C, CD117+ mast cell aggregates. 1-4 In patients with systemic … SB Mast cell leukemia is the leukemic variant of system ic mastocytosis (9741/3) in which bone marrow aspirate smears contain greater than or equal to 20% mast cell s. These mast cell s are usually … AS This reflects the hematopathologic practice of the presenters. et al. Oxford University Press is a department of the University of Oxford. Application of the defined diagnostic criteria can confirm or exclude mastocytosis in most cases. P Ann Oncol 2014;24(9):1691-1700. To be able to separate the different forms of SM, pathologists must be aware of clinical symptoms, especially the so-called B findings, referring to an organomegaly (hepatosplenomegaly and/or lymphadenopathy), and even C findings, indicating organ dysfunction due to widespread mast cell infiltration (eg, cytopenia and/or ascites in aggressive SM with strong infiltration of bone marrow and liver). 2.1. MR et al. At autopsy the bone marrow, spleen, liver, and lymph nodes were extensively infiltrated by atypical tumor cells with occasional bi‐ or multi‐lobated nuclei. The biopsy specimen should be fixed in 5% buffered formalin and acid-decalcified in EDTA overnight. et al. Horny He had no visible skin lesions. 14.2.2.5 Mast Cell Leukemia Mast cell leukemia is a rare condition accounting for less than 1% of mast cell disorders. Valent Valent F JH Outline the treatment and management options available for systemic mastocytosis. The unfixed splenectomy specimen weighed 1,736 g and measured 26 × 17.5 × 10 cm. Cutaneous mastocytosis … H (Case 43) A, Circulating hypogranulated mast cells in the peripheral blood smear (Wright-Giemsa). Bennett spleen, and esophagus. Hematopoiesis is intact. Parwaresch I et al. et al. Smoldering systemic mastocytosis is a variant of indolent systemic mastocytosis. Diaz-Agustin Mast cells are tissue-resident cells that express the stem cell factor receptor CD117 (KIT), the high-affinity IgE receptor (FcɛRI), and tryptase, but lack expression of CD34. 3. The diagnosis was SM with mild infiltration of bone marrow and marked involvement of the mucosa of the large bowel (and concurrent adenocarcinoma). et al. J Sotlar Cytologic: 2.1.1. An autopsy case of mast cell leukemia. Ruck Five cases were selected to demonstrate the broad spectrum of mastocytosis and possible difficulties in achieving a correct diagnosis. On the other hand, in SM-myeloproliferative neoplasm with eosinophilia (MPNEo), KITD816V is missed not only in the MPNEo but, very surprisingly, also in the SM, although compact infiltrates of CD25+ mast cells are present. CY et al. Hairy cell leukemia (HCL) is a rare mature B-cell neoplasm which involves bone marrow and spleen but generally only few circulating cells are seen on peripheral blood smears. C (d) CD117, but negative for (e) M Samorapoompichit Mast cells are a type of white blood cell that play an important role in helping the immune system defend the body from disease. F Despite this lack of myeloblasts, these cases should be considered acute leukemias. DD Copyright © 2021 American Society for Clinical Pathology. et al. Mast cells are multifunctional hematopoietic cells producing various proinflammatory mediators. Learn more. Here, a multifocal involvement of the bone marrow (between 1% and 20% of the section area) also is usually present, but in up to 10% of cases, SM is revealed only by using immunohistochemical analysis to detect even small or minute compact infiltrates obscured by the dominating hematologic neoplasms. M … The diagnosis of MCL requires the presence of SM criteria, accompanied by leukemic infiltrating of atypical mast cells (MCs) in bone marrow (BM), peripheral blood as well as extracutaneous organs. E Hall and coworkers. Sperr Homology of the rat basophilic leukemia cell and the rat mucosal mast cell. Although bone marrow is the main tissue for diagnosis of systemic mastocytosis (SM), the demonstration of compact mast cell infiltrates in extramedullary tissues like lymph node, spleen, mucosa, etc, should be regarded as indicative of involvement by the disease.7,8 Rarely, the diagnosis of mastocytosis is first established in the mucosa of the gastrointestinal tract or in the spleen, and involvement of the bone marrow is recognized in a second step when an adequate tissue sample is analyzed. Mast cell neoplasms comprise a clinically and biologically heterogeneous group of disorders. Sperr It is characterized by diffuse infiltration of the bone marrow by atypical and/or immature mast cells, and often presence of mast cells in the peripheral blood. The case involved a 42 year-old woman with dyspnea and a skin rash. A right hemicolectomy specimen contained a 5.5-cm mucinous adenocarcinoma at the cecum infiltrating the lamina muscularis propria (pT2). Bone marrow histologic examination revealed multifocal, dense, compact infiltrates of partially spindle-shaped mast cells, some adjacent lymphoid nodules, and an interstitial increase in eosinophils Image 4. Bone marrow histologic examination revealed diffuse and focal infiltration by atypical mast cells coexpressing tryptase and CD117, whereas myeloperoxidase was negative Image 3. Myelomastocytic leukemia versus mast cell leukemia versus systemic mastocytosis associated with acute myeloid leukemia: a diagnostic challenge Am J Hematol. C Stellmacher Blood and bone marrow smears yielded no significant dysplastic changes of hematopoietic cells and no increase in blast cells. Jordan The activating point mutation KITD816V of c-kit was detected in the colonic mucosa. Erika Mitsui. The patient was scheduled for splenectomy. H-P A 52‐year‐old woman presented with urticaria‐like … B Proposals of the Spanish Network on Mastocytosis (REMA) Luis Escribano, 1* Beatriz Diaz-Agustin, Antonio Lo´pez,2 … Fritsche-Polanz All of the following: 1. Reported herein is an autopsy case of mast cell leukemia, a rare form of systemic mastocytosis, complicated with portal hypertension. Bache Ribadeau Dumas Enter your email address below and we will send you your username, If the address matches an existing account you will receive an email with instructions to retrieve your username, By continuing to browse this site, you agree to its use of cookies as described in our, I have read and accept the Wiley Online Library Terms and Conditions of Use, https://doi.org/10.1111/j.1440-1827.2009.02451.x. K The following neoplastic disorders need to be included in the differential diagnosis of mastocytosis: Myelomastocytic leukemia25,26 represents an extremely rare type of myelogenous leukemia with prominent signs of mast cell differentiation but does not fulfill the criteria for mastocytosis; in particular, compact mast cell infiltrates and KITD816V are missing. The pattern of bone marrow … H-P Malignant hematopoietic cell lines: in vitro models for the study of mast cell leukemia. Seldin DC, Adelman S, Austen KF, Stevens RL, Hein A, Caulfield JP, Woodbury RG. P Several mast cells are present in this photograph. Aggressive SM always is accompanied by cytopenias. Kaiserling In aggressive SM,16 the bone marrow is markedly infiltrated by often confluent, dense sheets of mast cells (≥30% of the section). Pathologists need to be aware of the disease and its mimickers. Drexler HG, MacLeod RA. 4.1. Hans-Peter Horny, MD, Mastocytosis: An Unusual Clonal Disorder of Bone Marrow–Derived Hematopoietic Progenitor Cells, American Journal of Clinical Pathology, Volume 132, Issue 3, September 2009, Pages 438–447, https://doi.org/10.1309/AJCPPXHMN5CJOXHZ. alpha- and beta-tryptase genes encode serine proteases that are abundantly expressed by mast cells. Sotlar surfaces. T and you may need to create a new Wiley Online Library account. M Mastocytosis, an unusual disorder of bone marrow–derived, clonally transformed hematopoietic progenitor cells, exhibits a broad spectrum of clinical and morphologic features ranging from a self-limiting benign disorder (ie, juvenile cutaneous mastocytosis) to highly aggressive neoplasms like mast cell leukemia. The major diagnostic criterion for mastocytosis is focal compact tissue infiltrate predominantly composed of mast cells. R The differential diagnoses of aggressive SM include smoldering SM, SM-AHNMD, aleukemic MCL, myelomastocytic leukemia, and tryptase-positive AML. et al. Sotlar First, an elevated serum tryptase level does not count as a minor diagnostic criterion in cases with myeloid (non–mast cell) neoplasms, and, second, spindle-shaped morphologic features can only be used as a minor criterion in compact mast cell infiltrates (when the original description is strictly applied). At least 6 months + persistent. When the bone marrow of patients with long-standing adult-type cutaneous mastocytosis (mostly urticaria pigmentosa) is examined, it is found to be positive in more than 80% of cases.9 (2) SM with associated hematologic non–mast cell clonal disease (SM-AHNMD) is the most frequent diagnosis in patients without cutaneous disease but in whom there is a clinical suspicion of a hematologic neoplasm.10–12 When all cases of myeloid neoplasm, irrespective of the subtype (myelodysplastic syndrome [MDS], myelodysplastic/myeloproliferative neoplasm [MD/MPN], MPN, AML), are studied with antibodies against tryptase, CD25, and CD117, in up to 10%, the presence of SM (SM-AHNMD) is revealed. These cells may constitute 90% or more of the marrow elements. A Sillaber Features: Large cells -- 4-5 times the diameter of a small lymphocytes. Bernd Valent SM-AHNMD in itself is very heterogeneous. H-P (Case 167) A, Bone marrow biopsy specimen showing dense aggregates of partially spindle-shaped mast cells surrounded by a strongly hypercellular marrow with marked increase in eosinophils (H&E). Dysplasia. H-P For full access to this pdf, sign in to an existing account, or purchase an annual subscription. Early on there are typically no symptoms. Well-differentiated SM23 manifests as an exclusively round-cell type of the disease with compact multifocal infiltrates lacking CD25. The differential diagnoses of cutaneous mastocytosis include mast cell hyperplasia and indolent SM. Kapadia CLL: It is the most common mature B-cell leukemia in the Western hemisphere and makes up 30% of all leukemias.It mainly affects middle-aged and elderly patients with a male predominance. Kimura N(1), Shiraishi S, Mizunashi K, Ohtsu H, Kimura I. et al. It can be subclassified a number of ways. WV … Horny Underrecognized Patterns of High-Grade Squamous Intraepithelial Lesion on ThinPrep Preparations: Markers of Follicular Helper T Cells Are Occasionally Expressed in T-Cell or Histiocyte-Rich Large B-Cell Lymphoma, Classic Hodgkin Lymphoma, and Atypical Paracortical HyperplasiaA Diagnostic Pitfall For T-Cell Lymphomas of T Follicular Helper Origin. et al. Accordingly, the hematopoiesis is often markedly reduced or even exhibits signs of dysplasia. Yuji Nishikawa, MD, PhD, Department of Molecular Pathology and Tumor Pathology, Akita University Graduate School of Medicine, 1‐1‐1 Hondo, Akita 010‐8543, Japan. It is not possible to estimate the true frequency of mastocytosis for several reasons: (1) Indolent SM is the most frequent diagnosis. Walchshofer Marafioti Blast percentage <20%. 1 The most common of these are clinically indolent clonal mast cell proliferations … There is a difference between someone who is healthy, with mast cells that are functioning normally, and someone with a mast cell disease, whose mast cells may be activating inappropriately in response to triggers, or may also be proliferating and accumulating in organ tissues. It is important to be aware that the different forms of mastocytosis include various differential diagnoses. In contrast, the demonstration of dense, focal and/or diffuse MC infiltrates can be regarded as indicative of primary MC disease/mastocytosis. BAL can be de novo or secondary to previous cytotoxic therapy. The median survival is six months. To confirm the mast cell nature of a tryptase-positive round cell, immunostaining with anti-KIT (CD117) is strongly recommended in all questionable cases because basophils are always CD117–. Diffuse large B-cell lymphoma. Blood smears showed marked eosinophilia with a preponderance of atypical hypogranulated variants. Use of antibodies against tryptase, CD117 (KIT), and CD25 is recommended in every suspected case. In 31 of 46 cases, there was agreement between the diagnosis made by the submitter and by the panel. Horny Sotlar Mast cell sarcoma is often misdiagnosed because the presenting cells bear little resemblance to normal mast cells and spindle-shaped mast cells frequently seen in systemic mastocytosis. Mast cell leukemia (MCL) is a variant of systemic mastocytosis (SM) marked by a significantly shortened lifespan. A Mast cells were also mostly round and hypogranulated. et al. Diagnosis of MCL, however, can only be established when more than 20% atypical mast cells are found in bone marrow smears. 3 The cells of mast cell sarcoma more … In the absence of established systemic acute leukemia, confirming the diagnosis in cases of suspected myeloid LC can be difficult. Because the serum tryptase level is usually markedly elevated, it is possible to monitor the disease serologically. 90% of mast cell disease only affects the skin (edited to add: based upon estimates… Read More »Mast cell … Refined diagnostic criteria and classification of mast cell leukemia and myelomastocytic leukemia: a consensus proposal. The serum tryptase level was markedly elevated (>200 ng/μL). Hairy cell leukemia . CBC results were unremarkable. M An SM-AHNMD (SM-MDS) in such cases is difficult to assess or exclude. The term occult mastocytosis can also be used in rare cases of mastocytosis in which previously examined tissue is available for reevaluation. Main differential diagnoses include aggressive systemic mastocytosis (ASM), in particular ASM in transformation; mast cell leukemia; T+ … et al. JH Because the FIP1L1-PDGFR-α fusion gene is missing, a myeloproliferative neoplasm with eosinophilia (MPNEo; formerly chronic eosinophilic leukemia) can be ruled out (compare with case 199). (reticulin). Both compartments of the disease should be subcategorized. In this study, serum total tryptase leve … J Need >=10% abnormal. Search for other works by this author on: Diagnostic criteria and classification of mastocytosis: a consensus proposal: conference report of “Year 2000 Working Conference on Mastocytosis.”, WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, CD25 indicates the neoplastic phenotype of mast cells: a novel immunohistochemical marker for the diagnosis of systemic mastocytosis (SM) in routinely processed bone marrow biopsy specimens, Serum tryptase levels in patients with mastocytosis: correlation with mast cell burden and implication for defining the category of disease, Diagnosis of mastocytosis: general histopathological aspects, morphological criteria, and immunohistochemical findings, Histopathological and immunophenotypical aspects of mastocytosis, Indolent systemic mast cell disease in adults: immunophenotypic characterization of bone marrow mast cells and its diagnostic implication, Blood findings in generalized mastocytosis: evidence of frequent simultaneous occurrence of myeloproliferative disorders, Clinical and biologic diversity of leukemias occurring in patients with mastocytosis, Systemic mastocytosis with associated clonal haematological non–mast cell lineage diseases: a histopathological challenge, Systemic mastocytosis associated with chronic idiopathic myelofibrosis: a distinct subtype of systemic mastocytosis associated with a clonal hematological non–mast cell lineage disorder carrying the activating point mutations, Acute myeloid leukemia with t(8;21) associated with “occult” mastocytosis: report of an unusual case and review of the literature, The natural course of urticaria pigmentosa, Aggressive systemic mastocytosis and related mast cell disorders: current treatment options and proposed response criteria, Mast cell leukemia: report of a case and review of the literature, Mast cell sarcoma with tissue eosinophilia arising in the ascending colon, Temporary response of localized intracranial mast cell sarcoma to combination chemotherapy, A case of smouldering mastocytosis with high mast cell burden, monoclonal myeloid cells, and C-, A case of smouldering mastocytosis with peripheral blood eosinophilia and lymphadenopathy, A novel form of mastocytosis with a transmembrane c-, Myelomastocytic overlap syndromes: biology, criteria, and relationship to mastocytosis, Myelomastocytic leukemia: myeloid neoplasm characterized by partial differentiation of mast cell–lineage cells, Expression of mast cell tryptase by myeloblasts in a group of patients with acute myeloid leukemia, CHIC2 deletion, a surrogate for FIP1L1-PDGFRA fusion, occurs in systemic mastocytosis associated with eosinophilia and predicts response to imatinib mesylate therapy, The tryptase positive compact round cell infiltrate of the bone marrow (TROCI-bm): a novel histopathological finding requiring the application of lineage specific markers, Immunohistochemical characterization of mast cell disease in paraffin sections using tryptase, CD68, myeloperoxidase, lysozyme, and CD20 antibodies, Diagnostic utility of staining for tryptase in patients with mastocytosis, Diagnosis of mastocytosis: value of cytochemistry and immunohistochemistry, Immunohistochemical properties of bone marrow mast cells in systemic mastocytosis: evidence for expression of CD2, CD117/Kit, and bcl-xL, An unusual case of systemic mastocytosis associated with chronic lymphocytic leukaemia (SM-CLL), Spleen findings in generalized mastocytosis: a clinicopathologic study, Splenic mastocytosis: report of two cases and detection of the transforming somatic c-, Morphologic properties of neoplastic mast cells: delineation of stages of maturation and implication for cytological grading of mastocytosis, KITD816V-associated systemic mastocytosis with eosinophilia and FIP1L1/PDGFRA-associated chronic eosinophilic leukemia are distinct entities, © American Society for Clinical Pathology, How I Diagnose Anaplastic Large Cell Lymphoma.

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