Ectopic G-Csf Production by Malignant Plasma Cells in Patients with Diagnostic Criteria of Chronic Neutrophilic Leukemia

2013 ◽  
Vol 01 (01) ◽  
Author(s):  
Rodríguez-Medina C
Keyword(s):  
Diagnostic Criteria ◽  
Plasma Cells ◽  
Chronic Neutrophilic Leukemia ◽  
Csf Production

Does antrochoanal polyp present with epistaxis?

2009 ◽  
Vol 124 (5) ◽  
pp. 505-509 ◽  
Author(s):  
R H Sayed ◽  
E E Abu-Dief
Keyword(s):  
Diagnostic Criteria ◽  
Plasma Cells ◽  
Inflammatory Cells ◽  
Endoscopic Examination ◽  
Controlled Study ◽  
Histopathological Analysis ◽  
Antrochoanal Polyp ◽  
Microscopic Appearance ◽  
Recurrent Epistaxis ◽  
Computed Tomography Scanning

AbstractObjective:To compare the gross and microscopic appearance of antrochoanal polyps associated with recurrent epistaxis, with those with a more typical presentation.Design:Prospective, controlled study.Methods:All patients underwent clinical and endoscopic examination, computed tomography scanning, and examination under anaesthesia, in order to detect the gross diagnostic criteria for antrochoanal polyp. Histological findings on light microscopy were compared for polyps presenting with epistaxis versus those without. The number of predominant inflammatory cells in the corium was determined in both groups and statistically compared using the Studentt-test.Results:Recurrent epistaxis was a presenting symptom in 10/84 (11.9 per cent) patients with gross diagnostic criteria for antrochoanal polyp. Grossly, these patients' polyps had a reddish, vascular surface in parts. Histologically, these polyps showed a highly vascular stroma with multiple dilated blood vessels, the typical appearance of an angiomatous antrochoanal polyp. Thrombi at different stages of development were detected, with no infarcts. The remaining cases (88.1 per cent) had no history of epistaxis; histologically, these patients' polyps showed an oedematous connective tissue core with few inflammatory cells. Plasma cells were predominant in the angiomatous polyps, being significantly more prevalent than in the ordinary antrochoanal polyps (p < 0.00).Conclusions:It would appear that only angiomatous antrochoanal polyps present with epistaxis. Detection of the characteristic gross appearance of these polyps may help avoid unwanted surgery. Histopathological analysis confirms the diagnosis. A significantly increased number of plasma cells may be the underlying cause of the histological changes seen in angiomatous antrochoanal polyps.

scholarly journals Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan

2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Kazuichi Okazaki ◽  
Hisanori Umehara
Keyword(s):  
Diagnostic Criteria ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Serum Levels ◽  
Mikulicz’S Disease ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Biopsy Specimens ◽  
Organ Specific ◽  
Low Sensitivity

Recent studies suggest simultaneous or metachronous lesions in multiorgans characterized by elevated serum levels of IgG4 and abundant infiltration of IgG4-positive plasma cells with various degrees of fibrosis. Two Japanese research committees for IgG4-RD, one from fibrosclerosis (Okazaki team) and the other from lymph proliferation (Umehara team) supported by the “Research Program for Intractable Disease” of the Ministry of Health, Labor, and Welfare of Japan, have agreed with the unified nomenclature as “IgG4-RD” and proposed the comprehensive diagnostic criteria (CDC) for IgG4-RD. Validation of the CDC demonstrated satisfactory sensitivity for the practical use of general physicians and nonspecialists but low sensitivity in the organs to be difficult in taking biopsy specimens such as type1 autoimmune pancreatitis (IgG4-related AIP), compared with IgG4-related sialadenitis/dacryoadenitis (Mikulicz's disease) and IgG4-related kidney disease. Although the diagnostic criteria covering all IgG4-RD are hard to be established, combination with the CDC and organ-specific diagnostic criteria should improve sensitivity.

scholarly journals IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease

2021 ◽  
Vol 2021 ◽  
pp. 1-16
Author(s):  
Sylvia Drazilova ◽  
Eduard Veseliny ◽  
Patricia Denisa Lenartova ◽  
Dagmar Drazilova ◽  
Jakub Gazda ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Histological Finding ◽  
Good Prognosis ◽  
High Serum ◽  
Obliterative Phlebitis ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis

IgG4-related sclerosing cholangitis, a biliary manifestation of an IgG4-related disease, belongs to the spectrum of sclerosing cholangiopathies which result in biliary stenosis. It presents with signs of cholestasis and during differential diagnosis it should be distinguished from cholangiocarcinoma or from other forms of sclerosing cholangitis (primary and secondary sclerosing cholangitis). Despite increasing information and recently established diagnostic criteria, IgG4-related sclerosing cholangitis remains underdiagnosed in routine clinical practice. The diagnosis is based on a combination of the clinical picture, laboratory parameters, histological findings, and a cholangiogram. Increased serum IgG4 levels are nonspecific but are indeed a part of the diagnostic criteria proposed by the Japan Biliary Association and the HISORt criteria for IgG4-SC. High serum IgG4 retains clinical utility depending on the magnitude of elevation. Approximately 90% of patients have concomitant autoimmune pancreatitis, while 10% present with isolated biliary involvement only. About 26% of patients have other organ involvement, such as IgG4-related dacryoadenitis/sialadenitis, IgG4-related retroperitoneal fibrosis, or IgG4-related renal lesions. A full-blown histological finding characterized by IgG4-enriched lymphoplasmacytic infiltrates, obliterative phlebitis, and storiform fibrosis is difficult to capture in practice because of its subepithelial localization. However, the histological yield is increased by immunohistochemistry, with evidence of IgG4-positive plasma cells. Based on a cholangiogram, IgG-4 related sclerosing cholangitis is classified into four subtypes according to the localization of stenoses. The first-line treatment is corticosteroids. The aim of the initial treatment is to induce clinical and laboratory remission and cholangiogram normalization. Even though 30% of patients have a recurrent course, in the literature data, there is no consensus on chronic immunosuppressive maintenance therapy. The disease has a good prognosis when diagnosed early.

scholarly journals Histopathologic Overlap between Fibrosing Mediastinitis and IgG4-Related Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-7 ◽  
Author(s):  
Tobias Peikert ◽  
Bijayee Shrestha ◽  
Marie Christine Aubry ◽  
Thomas V. Colby ◽  
Jay H. Ryu ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Plasma Cells ◽  
Immunosuppressive Agents ◽  
Granulomatous Disease ◽  
Obliterative Phlebitis ◽  
Fibrosing Mediastinitis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Formalin Fixed Paraffin ◽  
Formalin Fixed Paraffin Embedded

Fibrosing mediastinitis (FM) and IgG4-related disease (IgG4-RD) are two fibroinflammatory disorders with potentially overlapping clinical and radiological features. In this paper, we looked for histopathologic features of IgG4-RD and enumerated infiltrating IgG4-positive plasma cells within mediastinal tissue biopsies from FM patients. We identified 15 consecutive FM surgical mediastinal tissue biopsies between 1985 and 2006. All patients satisfied the clinical and radiological diagnostic criteria for FM. All patients had either serological or radiological evidence of prior histoplasmosis or granulomatous disease, respectively. Formalin-fixed paraffin-embedded tissue sections of all patients were stained for H&E, IgG, and IgG4. Three samples met the predefined diagnostic criteria for IgG4-RD. In addition, characteristic histopathologic changes of IgG4-RD in the absence of diagnostic numbers of tissue infiltrating IgG4-positive plasma cells were seen in a number of additional cases (storiform cell-rich fibrosis in 11 cases, lymphoplasmacytic infiltrate in 7 cases, and obliterative phlebitis/arteritis in 2 cases). We conclude that up to one-third of histoplasmosis or granulomatous-disease-associated FM cases demonstrate histopathological features of IgG4-RD spectrum. Whether these changes occur as the host immune response against Histoplasma or represent a manifestation of IgG4-RD remains to be determined. Studies to prospectively identify these cases and evaluate their therapeutic responses to glucocorticoids and/or other immunosuppressive agents such as rituximab are warranted.

scholarly journals Smoldering (Asymptomatic) Multiple Myeloma: Current Diagnostic Criteria, New Predictors of Outcome, and Follow-Up Recommendations

2010 ◽  
Vol 28 (4) ◽  
pp. 690-697 ◽  
Author(s):  
Joan Bladé ◽  
Meletios Dimopoulos ◽  
Laura Rosiñol ◽  
S. Vincent Rajkumar ◽  
Robert A. Kyle
Keyword(s):  
Risk Factors ◽  
Multiple Myeloma ◽  
Diagnostic Criteria ◽  
Plasma Cells ◽  
Cell Mass ◽  
Cytotoxic Agents ◽  
Predictors Of Outcome ◽  
Skeletal Involvement ◽  
Delay Progression

Purpose To provide an overview on smoldering (asymptomatic) multiple myeloma (SMM) including current diagnostic criteria, predictors of progression, pattern of progression, and outcome. Design A comprehensive review of the literature on risk factors for progression, treatment attempts to delay progression and outcome in patients with SMM. Results The risk factors for progression of SMM include: plasma cell mass including M-protein size and percentage of bone marrow clonal plasma cells (BMPC), abnormal free light chain ratio, proportion of phenotypically abnormal BMPC, immunoparesis, evolution pattern (evolving v nonevolving), and pattern of magnetic resonance imaging abnormalities. Most patients with SMM progress with anemia and/or skeletal involvement. Immediate therapy with cytotoxic agents, such as melphalan/prednisone has not resulted in improved outcome. Patients should not be treated until progressive disease with end-organ damage occurs. Increasing anemia is the most reliable indicator of progression. Conclusion These recently recognized predictors of outcome may be helpful for better disease monitoring and for investigation of new treatment approaches. Thus, recommendations for follow-up every to 3 to 6 months depending on the risk of progression are suggested, and clinical trials with new noncytotoxic biologically derived agents to delay progression, particularly in high-risk patients, are ongoing.

scholarly journals Multiple myeloma: Looking beyond standards

2016 ◽  
Vol 02 (01) ◽  
pp. 023-028
Author(s):  
Esha Kaul ◽  
Sanjeev Sharma
Keyword(s):  
Multiple Myeloma ◽  
Diagnostic Criteria ◽  
Plasma Cells ◽  
Residual Disease ◽  
Early Stage ◽  
Treatment Options ◽  
Autologous Stem Cell Transplant ◽  
Cell Transplant ◽  
Frequent Relapses

AbstractMultiple myeloma has been regarded as an incurable disease with frequent relapses. The diagnostic criteria have been revised multiple times to include early stage of the disease where treatment can be effective and can prolong the survival. Newer diagnostic criteria for myeloma have incorporated ≥60% plasma cells in the bone marrow and serum free light chain ratio (involved to uninvolved free light chains) of ≥100. The role of positron emission tomography-computed tomography scans has been recognized, and it has been increasingly utilized upfront in the management of multiple myeloma. Role of minimal residual disease monitoring has been studied in multiple trials and will in near future guide the treatment. Autologous stem cell transplant is still the preferred consolidation therapy after initial three or four drug induction. With the use of novel drugs combinations and with emerging treatment options the standard of care of myeloma patients will change.

Hashimoto's Thyroiditis with Increased IgG4-Positive Plasma Cells: Using Thyroid-Specific Diagnostic Criteria May Identify Early Phase IgG4 Thyroiditis

Thyroid ◽  
2020 ◽  
Vol 30 (2) ◽  
pp. 251-261 ◽  
Author(s):  
Yaqiong Li ◽  
Xinli Wang ◽  
Zhiyan Liu ◽  
Jiwei Ma ◽  
Xiaoyan Lin ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Hashimoto’S Thyroiditis ◽  
Early Phase ◽  
Plasma Cells ◽  
Hashimoto's Thyroiditis

scholarly journals Treatment of Coexisting Chronic Neutrophilic Leukemia and Light Chain Multiple Myeloma with Hydroxyurea, Bortezomib, and Dexamethasone

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Evelyn Taiwo ◽  
Huiying Wang ◽  
Robert Lewis
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Plasma Cells ◽  
Complete Blood Count ◽  
Bone Marrow Aspiration ◽  
Normal Karyotype ◽  
Subdural Hemorrhage ◽  
Peripheral Blood Flow ◽  
Chronic Neutrophilic Leukemia ◽  
First Case

A 63-year-old female was incidentally found to have leukocytosis and referred to the hematology service for evaluation. Complete blood count (CBC) revealed neutrophilia with band predominance and mild thrombocytopenia. Peripheral blood flow cytometry was unremarkable without any evidence of lymphoproliferative disorder or myeloblasts. Bone marrow aspiration and biopsy revealed a markedly hypercellular marrow with myeloid lineage predominance and approximately 10% plasma cells. The monoclonal gammopathy was determined as lambda light chain with a kappa/lambda ratio of 0.06. Cytogenetics revealed normal karyotype, JAK2 kinase was negative, and rearrangement of BCR-ABL1, PDGFRA, PDGFRB, and FGFR1 was negative. The patient was diagnosed with chronic neutrophilic leukemia (CNL) associated with light chain multiple myeloma, complicated by a subdural hemorrhage. She was treated with hydroxyurea and bortezomib/dexamethasone and had complete response with normalization of CBC and kappa/lambda ratio. To the best of our knowledge, we report the first case of chronic neutrophilic leukemia and multiple myeloma treated with bortezomib/dexamethasone.

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