Rituximab, Endoxan, Ganciclovir, Dexamethasone, Tocilizumab Sequential Treatment In Kaposi's Sarcoma-Associated Herpesvirus (KSHV) -Related Multicentric Castleman Disease. A Case Report with 8 Months Follow-up

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4872-4872
Author(s):  
Francesco Iuliano ◽  
Tecla Mingrone ◽  
Stefania Infusino ◽  
Alessia Perricelli ◽  
Angelo Pomillo ◽  
...  
Keyword(s):  
Conflicts Of Interest ◽  
Sequential Therapy ◽  
Clinical History ◽  
Castleman Disease ◽  
High Grade Fever ◽  
Intravenous Ganciclovir ◽  
Multicentric Castleman Disease ◽  
First Case ◽  
History Of

Abstract Abstract 4872 Clinical history of Kaposi sarcoma-associated herpesvirus (KSHV),-related Multicentric Castleman Disease is characterized by a rapidly progressive and often fatal course. We report a case successfully treated sequentially with a course of 4 infusions of rituximab 375mg/m2 at weekly intervals, Endoxan, 750 mg/m2 as an intravenous infusion once weekly for 2 weeks, intravenous ganciclovir, 5 mg/kg twice daily for 2 weeks and then once daily, dexamethasone 20 mg on days 1–4, 9–12, 17–20 and anti-interleukin-6 receptor antibody (tocilizumab) administered intravenously at a dose of 8 mg/kg every 2 weeks, starting at day 32. On December 12, 2009, a 54-year-old man came to the medicine department because of a 2-week history of progressive fatigue, wasting, high-grade fever (39°C), profuse sweating, and severe autoimmune hemolytic anemia (4.2 mg/dL). He had been diagnosed with asymptomatic MCD 6 months before admission. Generalized lymphadenopathy and hepato-splenomegaly were evident on physical examination and enlarged lymph nodes in retroperitoneal regions (CT scan). Blood biochemistry showed very high CRP, IL-6, beta 2 microglobulin serum levels, low LDH and albumin, increase polyclonal immunoglobulin, grade 3 thrombocytopenia and neutropenia. Serologic tests (Epstein-Barr virus, hepatitis B and C viruses, cytomegalovirus and human immunodeficiency virus) were negative. On hospital day 10, 250.000 copies of HHV-8 DNA were detected in 1 mL plasma by RT-PCR. The patient achieved CR two months after admission, still maintained at 8 month follow-up. HHV-8 DNA became undetectable over the course of 8 weeks, at which time ganciclovir was discontinued. Conclusions: In our case the onset of the disease was serious and life-threatening. Sequential therapy has proven to be able to save the life of the patient acting on different biological targets. To date is the first case treated with this schedule. Disclosures: No relevant conflicts of interest to declare.

HAART Does Not Improve the Outcome of HIV-Related Multicentric Castleman Disease: Results of a Multicentric Retrospective Study

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4918-4918 ◽  
Author(s):  
Chiara Cattaneo ◽  
Emanuela Vaccher ◽  
Alessandro Re ◽  
Salvatore Casari ◽  
Erika Borlenghi ◽  
...  
Keyword(s):  
Median Time ◽  
Conflicts Of Interest ◽  
Primary Effusion Lymphoma ◽  
Case Series ◽  
Central Nervous System Lymphoma ◽  
Castleman Disease ◽  
Time Interval ◽  
Female Ratio ◽  
Multicentric Castleman Disease

Abstract Abstract 4918 Background. Multicentric Castleman Disease (MCD) is a rare lymphoproliferative disorder, strictly related to Kaposi Sarcoma (KS) as both associated to HHV-8 infection. Like other HIV-related diseases, such as Non Hodgkin Lymphoma (NHL) and Primary Central Nervous System Lymphoma, MCD prevalence is known to be increased in HIV-pos subjects. However, limited case series among this subset of patients (pts) are reported and data regarding epidemiological variations in the pre and post HAART era are often non conclusive. In order to evaluate possible differences between pre- and post-HAART era, we retrospectively evaluated epidemiological and clinical characteristics of all pts affected by HIV-pos MCD afferent to two Italian Institutions. Patients and Methods. Data concerning biological, clinical and prognostic factors of HIV-pos MCD pts were collected and reported on a database. Pts were grouped according to the date of diagnosis in pre- (before 1997) and post-HAART (after 1997) era. Results. During a 21-year period (1990-2011), 35 HIV-pos MCD pts were observed at our Institutions, nine in the pre- and 26 in the post-HAART era. Male/Female ratio was 30/5; median age 37y (23-65). Histological subtype in 34 evaluable cases was hyaline-vascular in 6, plasmacytic in 20 and mixed cellularity in 8. Median time interval from HIV-pos detection to MCD diagnosis was 24 months (range 0–157) and CD4 count at MCD diagnosis 233/mcL (26-839). All these MCD baseline characteristics were not statistically different between pre- and post-HAART era. A concomitant diagnosis of KS was made in 18/35 (51.4%) cases, all but one in the post-HAART era. NHL was diagnosed concurrently with MCD in 2/35 (5.7%) pts (1 Primary Effusion Lymphoma, PEL, and 1 Plasmablastic Lymphoma, PBL); in 8/35 (22.8%) cases NHL developed after MCD diagnosis (2 PEL, 1 PBL, 2 Diffuse Large Cell, DLC, 1 unspecified, respectively). Median time from MCD to NHL was 19 mo (0-71). Evolution toward NHL was observed in 3 (33%) cases in the pre-HAART era and in 5 (19%) in the post-HAART era (p=0.39, Fisher's exact test). Six pts did not receive any type of treatment, 6 were treated with HAART only and 23 with different therapies, including antivirals, steroids, chemotherapy and rituximab (alone in 1 pt, in combination with chemotherapy in 5). Nineteen/23 pts received HAART together with other therapies. Two pts treated with rituximab developed NHL (1 PEL and 1 DLC). A complete or partial radiological response, together with clinical improvement was observed in 19/25 of evaluable pts (76%). Thirty pts were evaluable for relapse/progression, mainly in the post-HAART era. Overall, 19/30 pts showed MCD progression or transformation to NHL; median PFS was 15 months. Nineteen pts died and 5 were lost to follow-up. Overall survival (OS) of the entire series was 28 months, without significant differences between pre and post-HAART era (18 and 28 months, OR 0.643 [CI 0.2406–1.045], median follow-up 18 and 9 months, respectively). Causes of death were evaluable in 18 cases: NHL (7), MCD (6), opportunistic infections (1), liver cyrrhosis (1), acute myocardial infarction (1), KS (1) and therapy-related toxicity (1). NHL and MCD were the most frequent cause of death in the post-HAART era (4 and 5 of the 10 cases, respectively). Although no differences in OS between MCD pts without or with NHL were seen (19 vs 28 months, OR 0.6786 [CI 0.2763–1.081], median follow-up 13 and 23 months, respectively), eight/10 pts with NHL died, in comparison with 11/21 pts without NHL evaluable for outcome and median time from NHL diagnosis to death was 2 months (0-31). Conclusions. Our data confirm that the prognosis of HIV-related MCD remains poor even after the advent of HAART. Unlike other lymphoproliferative disorders, HAART did not impact on outcome of HIV-related MCD, suggesting that MCD can “escape” immune reconstitution. A concomitant diagnosis of NHL and uncontrolled MCD seem to be the main reason for an unfavourable outcome, particularly in the post-HAART era. New therapeutic approaches, including rituximab, should therefore aim at avoiding NHL transformation and controlling “MCD-related cytokine storm”. Disclosures: No relevant conflicts of interest to declare.

Protective Effect of Rituximab Against Lymphoma Risk In HIV-Associated Multicentric Castleman Disease

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2869-2869
Author(s):  
Jean Marie Michot ◽  
Laurence Gérard, MD ◽  
Claire Fieschi ◽  
Sarah Burcheri ◽  
Eric Oksenhendler ◽  
...  
Keyword(s):  
Overall Survival ◽  
Castleman Disease ◽  
Control Group ◽  
First Line ◽  
Multicentric Castleman Disease ◽  
Median Period ◽  
History Of ◽  
Lymphoma Risk

Abstract Abstract 2869 Human Immunodeficiency Virus (HIV)-associated multicentric Castleman disease (HIV-MCD) is a rare lymphoproliferative disorder occurring in patients co-infected with HHV-8. Long-term prognosis remains severe mainly because of the high incidence of aggressive HHV-8 associated non-Hodgkin lymphoma (NHL). Chemotherapy is usually the first line therapy in active HIV-MCD. Most patients remain dependent on chemotherapy. Recent studies have demonstrated that rituximab allows to resume chemotherapy in most patients. However, effect of rituximab on long-term incidence of lymphoma is unknown. All consecutive patients with HIV-MCD from the national HIV-lymphoproliferative cohort (Clinical site, Saint-Louis Hospital, Paris, France) were enrolled in combination antiretroviral therapy (cART) era. Patients with NHL within the 2 months prior or following rituximab were excluded from analysis. Patients treated with rituximab received 4 infusions, 375 mg per m2 at weekly intervals. Primary objective of the study was to compare incidence of NHL between patients who received rituximab (rituximab group) and who did not received rituximab (control group). Secondary objectives were to compare overall survival and to describe tolerance to rituximab. Survival and NHL incidence was estimated using Kaplan-Meier method and tested with log-rank test. 115 patients with HIV-MCD were included in the HIV-lymphoproliferative cohort until november, 2009. Patients were followed for a mean period of 3 years. 7 patients received rituximab as part of LNH therapy and were excluded from analysis. 33 patients (29%) received rituximab as part of MCD therapy. In rituximab group at MCD diagnosis, mean age was 41 years, 24 patients (73%) received antiretroviral therapy, HIV viral load was undetectable (< 500 c/ml) in 10 patients (30%), median CD4 cell count was 233 × 106/L [IQR, 101–368], median nadir CD4 was 160 × 106/L [IQR, 61–194], 14 patients (42%) had an history of Kaposi sarcoma (KS) before rituximab therapy, which was in complete remission or stable at time of rituximab therapy. All 33 patients received chemotherapy as first line MCD treatment: etoposide (n=27), vinblastine (n=4), anthracyclines (n=2). The median period from MCD diagnosis to rituximab therapy was 7.7 months [IQR, 4.6–23.8]. All but 2 patients received the 4 scheduled infusions. These 2 patients discontinued rituximab after the second infusion; one died at day 15 from progressive MCD and the other one discontinued for investigation of rectal tumor. 4 patients received a second course of 4 rituximab infusions for MCD relapse, after a median period of 19.5 months (IQR, 15 to 23). The 3-years probability of developing lymphoma was 0.04% [95%CI,0.01-0.99] in rituximab group (n=33) and 23% [95%CI,14-34] in control group (n=74) (P=0.001). The only lymphoma in rituximab group occurred 22 months after rituximab therapy and patient died. In rituximab group, the median overall survival was 15.7 years compared to 5.2 years in control group (P=0.0007). During total follow-up, 5 patients (15%) died in rituximab group and 28 patients (38%) died in control group. Causes of death in rituximab group was MCD attack (n=1), lymphoma (n=1), acute myeloid leukemia (n=1), infection (n=1), cardiovascular disease (n=1). No severe adverse event was observed during rituximab infusions. Within the first year following rituximab therapy, 9 patients (27%) developed mild to moderate infections; pneumonia (n=2), herpes reactivation (n=2), enterocolitis (n=2), dacryocystitis (n=1), folliculitis (n=1), staphylococcus catheter-related infection (n=1). Transient grade 3 neutropenia occurred in 2 patients (6%). Exacerbation of KS was observed in 4 out of the 15 patients with prior history of KS (4, 4, 7, 36 months after rituximab therapy, respectively), and required specific treatment in 2 patients. Despite improvement in overall survival of HIV-MCD in cART era, risk for developing lymphoma remains high (23% at 3 years). Rituximab seems to induce long-term protection against lymphoma risk, with only one patient developing lymphoma during the 3-year follow-up period in our cohort. Disclosures: No relevant conflicts of interest to declare.

scholarly journals The rare hemoglobin variant Hb Mizuho: report of a Swiss family and literature review

2021 ◽  
Author(s):  
Linet Njue ◽  
Cesare Medri ◽  
Peter Keller ◽  
Miriam Diepold ◽  
Behrouz Mansouri Taleghani ◽  
...  
Keyword(s):  
Literature Review ◽  
Hemolytic Anemia ◽  
De Novo ◽  
Clinical History ◽  
Molecular Techniques ◽  
De Novo Mutation ◽  
First Case ◽  
History Of ◽  
Transfusion Dependency ◽  
Unstable Hemoglobin

AbstractHb Mizuho is a very rare unstable hemoglobin; here, we describe the clinical history of three Swiss family members with Hb Mizuho together with a systematic review of the previously six published cases. The clinical history of the adult woman we report here is unique since this is the first Hb Mizuho presenting with Moyamoya complications and the first case reported with long-term erythrocyte exchange. The literature review showed that Hb Mizuho was mainly reported as a de novo mutation, with the exception of children descended from known cases. All published patients with this unstable hemoglobin showed severe hemolytic anemia with the exception of one; all were regularly transfused. Patients with higher HbF levels might require fewer transfusions. All patients underwent splenectomy at a median age of 4 years and had variable clinical improvement; some achieved complete resolution of transfusion dependency after splenectomy. Iron overload in Hb Mizuho patients seems to be mainly attributed to transfusions and has less to do with ineffective erythropoiesis. Diagnosis might be challenging; a normal hemoglobin electrophoresis should not rule out the diagnosis of unstable hemoglobin in patients with otherwise unexplained hemolytic anemia. This series shows the enormous utility of using molecular techniques for diagnosis.

Low-Grade Mucosa-Associated Lymphoid Tissue Lymphoma Involving the Kidney: Report of 3 Cases and Review of the Literature

2006 ◽  
Vol 130 (1) ◽  
pp. 86-89 ◽  
Author(s):  
Libo Qiu ◽  
Pamela D. Unger ◽  
Robert W. Dillon ◽  
James A. Strauchen
Keyword(s):  
Lymphoid Tissue ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Lymphoid Cells ◽  
Low Grade ◽  
The Third ◽  
First Case ◽  
Abundant Cytoplasm ◽  
History Of

Abstract Low-grade B-cell lymphoma of mucosa-associated lymphoid tissue involving the kidney is rare. We report a series of 3 cases. The first case occurred in an 83-year-old woman who presented with back pain. The second case was a 53-year-old man with a history of sarcoidosis who was found, in the course of evaluation of sarcoidosis, to have a right renal mass. The third case occurred in a 72-year-old man who had a history of periorbital mucosa–associated lymphoid tissue lymphoma and had been treated with surgery and radiation 1 year prior to this presentation. Histologically, all 3 patients showed infiltrate of uniform small-to-medium–sized lymphocytes with irregular nuclear contours and abundant cytoplasm resembling centrocytes or monocytoid lymphoid cells. The first patient received chemotherapy without complications. The second patient underwent a partial nephrectomy and was asymptomatic at the subsequent follow-up. The third patient developed a pulmonary embolism following nephrectomy, and further follow-up is not available.

scholarly journals Synchronous cytomegalovirus infection in a newly diagnosed ulcerative colitis patient

2017 ◽  
Vol 8 (1) ◽  
Author(s):  
Jin Yu Chieng ◽  
Yasotha Sugumaran ◽  
Sellymiah Adzman ◽  
Pan Yan
Keyword(s):  
Ulcerative Colitis ◽  
Medical Illness ◽  
C Reactive Protein ◽  
Colitis Patient ◽  
Intravenous Ganciclovir ◽  
Reactive Protein ◽  
Chronic Medical Illness ◽  
History Of ◽  
Cmv Colitis

A 61-year-old Punjabi female patient presented with six months history of mild abdominal discomfort with bloody diarrhea. She did not have underlying chronic medical illness; she neither took steroid nor immunosuppressant. She was found anemic, thrombocytosis, and elevated C-reactive protein. Colonoscopy showed moderate left sided colitis, with histopathology evidence of ulcerative colitis (UC) with cytomegalovirus (CMV) infection. Her serum anti-CMV IgM antibody was detected. She was treated with intravenous ganciclovir, together with 5-ASA and tapering dose of steroid. Anemia was corrected. Subsequent clinic reviews and follow up endoscopies showed dramatically improvement. CMV colitis should be considered for the patients presenting with moderate to severe UC. Early prescription of antiviral would be beneficial in the treatment of flare of UC.

The Small Blue Cell Dilemma Associated With Tamoxifen Therapy

2001 ◽  
Vol 125 (8) ◽  
pp. 1047-1050 ◽  
Author(s):  
Yi Jun Yang ◽  
Linda K. Trapkin ◽  
Roberta K. Demoski ◽  
Jeannette Bellerdine ◽  
Celeste N. Powers
Keyword(s):  
Older Patients ◽  
Endometrial Hyperplasia ◽  
Clinical History ◽  
Tamoxifen Therapy ◽  
Tamoxifen Treatment ◽  
Endometrial Polyps ◽  
High Incidence ◽  
History Of ◽  
Blue Cell

Abstract Context.—Several endometrial diseases, such as endometrial hyperplasia, endometrial carcinoma, and endometrial polyps, have been reported to be associated with tamoxifen administration. We recently observed a high incidence of distinctive small blue cells in Papanicolaou tests of women who had received tamoxifen treatment for breast carcinoma. Objectives.—To define the characteristics of these small blue cells, to identify the patient population in which they are found, and to determine the clinical significance and possible etiology of these findings. Design.—A total of 154 Papanicolaou tests from 60 patients with a clinical history of tamoxifen therapy were reviewed retrospectively. Results.—Small blue cells were found in 40% of Papanicolaou tests from patients who received tamoxifen therapy. Patients with small blue cells in their Papanicolaou tests were an average of 9 years older at the time tamoxifen therapy was initiated than those without. Among the available follow-up surgical biopsies, no malignant diagnoses were made. Conclusions.—We conclude that these distinctive small blue cells are found more frequently in older patients and most probably represent proliferative reserve cells of cervical/vaginal epithelium resulting from the estrogenic agonist effect of tamoxifen. More importantly, they are nonneoplastic in nature.

Revising Recommendations and Outcome Measurements after Complex Open Abdominal Wall Reconstruction

2015 ◽  
Vol 81 (10) ◽  
pp. 955-960 ◽  
Author(s):  
Shawn Diamond ◽  
H. Gill Cryer
Keyword(s):  
Abdominal Wall ◽  
Abdominal Wall Reconstruction ◽  
Clinical History ◽  
Long Term Results ◽  
Functional Domain ◽  
Grading Systems ◽  
History Of ◽  
Grade Ii ◽  
Restricted Use

Grading systems developed by the Ventral Hernia Working Group (VHWG) for complex open abdominal wall reconstruction rely on limited outcomes: surgical site occurrence (SSO) and hernia recurrence. This does not account for the longitudinal restoration of a functional abdominal wall and the ability to correct complications. We performed a single-site, retrospective review of consecutive complex open abdominal wall reconstruction interventions with 24-month minimum follow-up to establish reoperation rates and compare long-term results to the VHWG. About 125 midline hernia repairs (>200 cm2) were studied. All had loss of functional domain and 47-month average follow-up. Demographics included: mean age 57 years, 47 per cent male, 63 per cent obese, and 34 per cent with contamination. Rates of SSO per VHWG grade were 9 per cent grade I, 45 per cent grade II, and 55 per cent grade III. Forty-three of 59 patients who developed complications were eventually successful after reoperation leading to an 87 per cent restoration rate. Select factors independently associated with reoperation included biological mesh and clinical history of infection. Although rates of SSO were higher than the VHWG published, we experienced high salvage rates except in patients who underwent biologic repair. We recommend restricted use of biologic mesh in contaminated and clean fields as well as modifications to the VHWG grading and recommendations.

scholarly journals A Rare Case of HHV-8 Associated Hemophagocytic Lymphohistiocytosis in a Stable HIV Patient

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Nonso Osakwe ◽  
Diane Johnson ◽  
Natalie Klein ◽  
Dalia Abdel Azim
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Rare Case ◽  
Viral Infections ◽  
Rare Condition ◽  
Lymph Node Biopsy ◽  
Castleman Disease ◽  
Human Herpes Virus 8 ◽  
Case Presentation ◽  
Multicentric Castleman Disease ◽  
History Of

Background. Hemophagocytic lymphohistiocytosis (HLH) is a rare condition associated with viral infections including HIV. Cases have been reported mainly in advanced HIV/AIDS. This is a rare case that reports HLH associated with human herpes virus-8 (HHV-8) associated multicentric Castleman disease in a stable HIV patient. Case Presentation. A 70-year-old Asian male patient with history of stable HIV on medications with CD 4 cell count above 200 presented with cough and fever and was initially treated for pneumonia as an outpatient. Persisting symptoms prompted presentation to the hospital. The patient was found to have anemia which persisted despite repeated transfusion of packed red cells. A bone marrow biopsy to investigate anemia revealed hemophagocytosis. A CT scan revealed multiple enlarged lymph nodes and hepatosplenomegaly. An excisional lymph node biopsy revealed HHV-8 associated multicentric Castleman disease. The patient deteriorated despite initiation of treatment. Conclusion. HLH can occur at any stage of HIV, rapid diagnosis to identify possible underlying reactive infectious etiology and prompt initiation of treatment is crucial to survival.

scholarly journals Metastatic Pancreatic Cancer with BRAF and P53 Mutations: Case Report of Therapeutic Response to Doublet Targeted Therapy

2020 ◽  
Vol 13 (3) ◽  
pp. 1239-1243
Author(s):  
Shenthol Sasankan ◽  
Lorraine Rebuck ◽  
Gloria Darrah ◽  
Moises Harari Turquie ◽  
Ian Rabinowitz
Keyword(s):  
Pancreatic Cancer ◽  
Case Report ◽  
Targeted Therapy ◽  
Therapeutic Response ◽  
Case Reports ◽  
Clinical History ◽  
P53 Mutation ◽  
Metastatic Pancreatic Cancer ◽  
First Case ◽  
History Of

We report on the clinical history of a 49-year-old female with metastatic pancreatic cancer. She was initially treated with standard chemotherapy as per current guidelines. She was found to have both a BRAF and P53 mutation, and received dabrafenib and trametinib with deep responses, both radiographically and biochemically (CA19-9). Her response has been more clinically relevant than responses in previous case reports of patients with BRAF-positive pancreatic cancer treated with targeted therapy. To the best of our knowledge, this is the first case report showing a dramatic therapeutic response to combination therapy with dabrafenib and trametinib in metastatic pancreatic cancer.

Natural History of 656 Patients with Non IgM MGUS.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4860-4860
Author(s):  
Carolina Nobile ◽  
Maria T. Petrucci ◽  
Francesco Bartolozzi ◽  
Anna Levi ◽  
Marianna De Muro ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Natural History ◽  
Clinical History ◽  
Cumulative Probability ◽  
Rapid Progression ◽  
Female Ratio ◽  
Predictive Parameters ◽  
Risk Of Progression ◽  
History Of

Abstract A monoclonal gammopathy of undetermined significance (MGUS) occurs in about 1% of the population over 50 years of age. Of these, about 20% evolves in Multiple Myeloma (MM); however, so far, predictive parameters of progression have not yet been identified.The aim of this study was to analyse the natural history of a cohort of non IgM MGUS and to identify whether or not there were laboratory parameters at diagnosis which can be utilized as prognostic markers of stable MGUS or progression to MM. From February 1974 to July 2001, 656 non IgM MGUS, whose clinical history was concluded (lost to follow up or died), have been followed at the Hematology of the University “La Sapienza” in Rome. The duration of follow up ranged from 2 months to 324 months, male/female ratio was 1.14, median age was 65 years (range 19–92). In each patient we evaluated: hemoglobin, platelet count, serum protein electrophoresis, serum concentration of monoclonal protein, serum calcium, creatinine, uric acid, BUN and percentage of bone marrow plasma cells.A monoclonal component (MC) of IgG type was documented in 543 patients (83%) while in 106 (16%) it was of IgA type, 6 patients had biclonal MC and 1 had a λ light chain MC; BJ proteinuria was detected in 78 (11%) patients at diagnosis. After a median follow up of 60.1 months (range 2–324) the MC remained stable in 496 patients (75%), whereas in 160 cases (25%) increased to evolve in MM. According to the literature, cumulative probability of progression to MM was 3%, 7% and 17% at 5, 10 and 15 years respectively. Differently from what observed by other investigators, in this cohort of pts, the MGUS of IgA type was not associated with a higher risk of progression to MM. The median time of progression to MM was 60.7 months (range 3–256) and factors associated with a more rapid progression to MM were advanced age and a higher number of bone marrow plasmacells. At diagnosis of MM, the concentration of the serum MC was significantly higher (P<0.003) in patients who evolved from MGUS than in those with a newly diagnosed MM. None of the studied parameters at diagnosis of MGUS were predictive of evolution to MM even though, levels of MC <2.4 g/dl and bone marrow plasmacell infiltration <9% indicated a slower progression to overt MM.

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