A resistant case of pyoderma gangrenosum with monoclonal gammopathy of unknown significance showing excellent response to combination therapy of methyl prednisolone pulse and oral cyclosporine

2016 ◽  
Vol 36 (2) ◽  
pp. 60
Author(s):  
NajmusS Jamadar ◽  
Saswati Das ◽  
AlokeK Roy
2015 ◽  
Vol 81 (2) ◽  
pp. 213 ◽  
Author(s):  
SarveshS Thatte ◽  
AtulM Dongre ◽  
SiddhiB Chikhalkar ◽  
UdayS Khopkar

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Marja Kovala ◽  
Minna Seppala ◽  
Kati Kaartinen ◽  
Seppo Meri ◽  
Eero Olavi Honkanen ◽  
...  

Abstract Background and Aims Monoclonal gammopathy is an entity where a B-cell or plasma cell clone produces monoclonal immunoglobulin. When paraproteinemia and a kidney disease is discovered without criteria for treatment of haematological malignangy, the entity is called monoclonal gammopathy of unknown significance (MGUS) or of renal significance (MGRS). It can cause variable histology, among which membranoproliferative glomerulonephritis (MPGN) has been described. Direct entrapment of paraprotein in glomeruli/tubules can be observed by immunofluorescence (IF), but IF can also be negative as paraprotein can cause complement-associated disease by acting as an activator of the classical pathway or as a dysregulator of the alternative pathway. Electron microscopy (EM) is needed to differentiate possible organized deposits. Method We investigated the prevalence, clinical parameters, histology, and the type of monoclonal gammopathy in biopsy-proven MPGN between 2006-2017. A total of 15 adult patients with a detected urine and/or serum paraprotein with concurrent biopsy-proven diagnosis were discovered among 60 patients (Figure 1). Two diagnostic biopsies were from transplants. Results MGUS was diagnosed in 15/60 (25%) of patients. Clinical variables are summarized in Table 1. The mean age at presentation was 59 years (37-79), 47-% were males. Smoldering myeloma was diagnosed in 2 (13%) patients and overt malignancy in 3 (20%) patients. Histological features are summarized in Table 2. There were 7 (47%) with dominant staining for C3 (6 with C3 glomerulonephritis, 1 Dense Deposition Disease) and 8 (53%) with dominant staining for Ig, of which 4 (31%) had mesangioproliferative, 4 (31%) membranoproliferative, 3 (23%) minimal change, 2 (15%) crescentic and 1 (8%) exudative pattern in light microscopy (LM). Seven (47%) biopsies, which did not stain for kappa or lambda light chains. The most common EM deposit location was subendothelial (69%). Conclusion MPGN was associated with a significant risk of underlying monoclonal gammopathy, as many (25%) patients were diagnosed with concurrent MGUS. When MPGN is observed, it should prompt investigations of the possible underlying monoclonal gammopathy and possibly, hematological neoplasm.


2013 ◽  
Vol 6 (4) ◽  
pp. 445-446
Author(s):  
Marsela Resuli ◽  
Finn Thomsen Nielsen ◽  
Peter Gimsing ◽  
Claus B. Andersen ◽  
Martin Egfjord

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4755-4755
Author(s):  
Lucie Kovarova ◽  
Ivana Buresova ◽  
Ludek Pour ◽  
Renata Suska ◽  
Zdenek Adam ◽  
...  

Abstract Introduction: Multiple myeloma (MM) is a B-cell neoplasia caused by the proliferation of clonal plasma cells (PCs). MM and benign monoclonal gammopathy of unknown significance (MGUS) are routinely distinguished on the basis of paraprotein concentration, level of PCs infiltration and the presence or absence of other clinical features. Flowcytometric detection of PCs according to the expression of CD38 and CD138 has limitation in discrimination between normal and abnormal PCs, but this is possible in multicolor phenotypic analysis. The aim of this study is to compare the numbers of normal and abnormal PCs in MGUS and MM subjects and to find some parameter useful for evaluation PCs distribution. Materials and methods: 51 newly diagnosed untreated MM patients (63,9±10,0 years old) and 31 non-treated MGUS subjects (64,2±13,8 years old) were analysed. Lysed whole bone marrows were analysed by flowcytometric immunophenotyping and PCs were indentified by expression of CD38, CD138, CD45 and also CD56 and CD19. Results: Discrimination between normal CD19+ PCs and abnormal CD56+ PCs was done on CD38+CD138+ population. Ratio of normal PCs count and abnormal PCs counts (normal/abnormal=N/A) was used to describe a distribution of PCs. Subjects with MGUS had 0,97±1,65% (average±SD) of CD38+CD138+ cells (median 0,45; range 0,03–7,47%) with average N/A ratio 2,91±3,94 (median 1,36; range 0,01–18,44). Newly diagnosed MM patients had 4,96±9,94% of CD38+CD138+ cells (median 0,85; range 0,02–41,80%) with average N/A ratio 1,70±3,03 (median 0,13; range 0–11,5). In 9,7% MGUS subjects evaluation of distibution of PCs showed transformation of MGUS into MM. In some newly diagnosed MM patients (31,4%) CD38+CD138+ plasma cells were positive for CD19 although they were aberrant and these PCs were mostly CD45+. In some patients (9,7% of MGUS; 17,6% of MM) PC did express neither CD19 nor CD56 and this fact may complicate further evaluation of PCs. Conclusions: Results confirmed that in MGUS subjects we can find lower numbers of PCs which are mostly CD45+CD19+. A majority of plasma cells in newly diagnosed MM patients are abnormal CD56+ PCs and these plasma cells are usually CD45−. Further follow-up of patients can confirm the N/A ratio as a predictive factor for transformation of MGUS into MM and its value for evidence of relapse or progression to active myeloma.


2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 8103-8103
Author(s):  
Hady Ghanem ◽  
Swathi Namburi ◽  
Gabriel Rivera ◽  
Richard Amdur ◽  
Geraldine P. Schechter

8103 Background: Monoclonal Gammopathy of Unknown Significance (MGUS) is characterized by the presence of a monoclonal immunoglobulin in the serum or the urine with no evidence of hematologic malignancy. A possible relationship between MGUS and increased incidence of NHM has been suggested in Caucasian populations. However, data in African Americans with MGUS are lacking. Methods: Non-MGUS controls were selected randomly from patients who did not have a paraprotein detected on electrophoresis (NMGUS) and were matched 2-to-1 to MGUS cases. Descriptive statistics and comparisons are presented to compare MGUS and NMGUS groups. Results: 492 male patients with MGUS patients were matched with 984 male NMGUS patients. 451 patients had abnormal serum protein studies (91.6%) and 40 had light chain disease (8.4%). Median age at diagnosis of MGUS was 68 years (28-81). 144 MGUS patients (29.2%) and 296 NMGUS patients (30%) had 1 or more NHM. The median age of diagnosis of 1st NHM was 70 (25-94) in the MGUS group and 68.4 in the NMGUS group (34.5-94.4). 19 MGUS patients (3.8%) and 27 NMGUS patients had 2 different types of NHM (2.7%). 1 MGUS patient (0.2%) and 3 NMGUS patients (0.3%) had 3 NHM. 57 patients had MGUS before NHM (11.5%) and 69 patients were diagnosed with MGUS after the diagnosis of NHM (14%), and median differences between diagnosis of MGUS and 1st NHM were 4 years (1-12 years) and 5 years (1-38 years) respectively. Types of NHM were comparable, and prostate cancer was the most prevalent NHM in both groups (15% of MGUS patients and 17% of NMGUS patients). Median time of follow up was 49.3 months for MGUS patients and 35.2 months for NMGUS patients 140 of the MGUS patients (28.4%) and 214 non-MGUS patients (21.75%) had died at data cut-off. Conclusions: Based on these observational data, prevalence and types of NHM appear to be comparable in MGUS and NMGUS African American patients. All cause mortality appears to be higher for NHM patients if they had MGUS. This pattern will need to be verified prospectively in a larger group of patients.


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