Clinical characteristics and progression risk factors for patients with monoclonal gammopathy of undetermined significance / 中华血液学杂志

Objective: To analyze the clinical presentation and progression risk factors of patients with monoclonal gammopathy of undetermined significance (MGUS) in China. Methods: We retrospectively assessed the clinical features and disease progression of 1 037 patients with monoclonal gammopathy of undetermined significance between January 2004 and January 2022 at Peking Union Medical College Hospital. Results: A total of 1 037 patients were recruited in the study, including 636 males (63.6%) , with a median age of 58 (18-94) years. The median concentration of serum monoclonal protein was 2.7 (0-29.4) g/L. The monoclonal immunoglobulin type was IgG in 380 patients (59.7%) , IgA in 143 patients (22.5%) , IgM in 103 patients (16.2%) , IgD in 4 patients (0.6%) , and light chain in 6 patients (0.9%) . 171 patients (31.9%) had an abnormal serum-free light chain ratio (sFLCr) . According to the Mayo Clinic model for risk of progression, the proportion of patients in the low-risk, medium-low-risk, medium-high risk, and high-risk groups were 254 (59.5%) , 126 (29.5%) , 43 (10.1%) , and 4 (0.9%) , respectively. With a median follow-up of 47 (1-204) months, 34 of 795 patients (4.3%) had disease progression, and 22 (2.8%) died. The overall progression rate was 1.06 (0.99-1.13) /100 person-years. Patients with non-IgM MGUS have a markedly higher disease progression rate per 100 person-years than IgM-MGUS (2.87/100 person-years vs 0.99/100 person-years, P=0.002) . The disease progression rate per 100 person-years in non-IgM-MGUS patients of Mayo classification low-risk, medium-low risk and medium-high risk groups were 0.32 (0.25-0.39) /100 person-years, 1.82 (1.55-2.09) /100 person-years, and2.71 (1.93-3.49) /100 person-years, which had statistically difference (P=0.005) . Conclusion: In comparison to non-IgM-MGUS, IgM-MGUS has a greater risk of disease progression. The Mayo Clinic progression risk model applies to non-IgM-MGUS patients in China.

Monoclonal gammopathy of undetermined significance clinic during the coronavirus disease-19 pandemic: caring for the vulnerable in an academic medical center

Background: Patients with monoclonal gammopathy of undetermined significance (MGUS) have clinical features including older age, presence of medical comorbidities, susceptibility to infections, and thrombotic tendencies which are relevant when assessing their risk during the coronavirus disease (COVID-19) pandemic. Objective: To study the vulnerability of patients with MGUS during the COVID-19 pandemic, we assessed the local management of MGUS patients and their clinical outcomes. Methods: Retrospective chart reviews were performed for all patients with MGUS seen at a university medical center clinic (2014-2020). Results: A total of 228 MGUS patients were included; 211 patients are alive, 7 patients died before the pandemic, and 10 patients died since the pandemic declaration. The mean age and the overall survival (OS) of the patients who died before versus during the pandemic were 83.0 versus 75.2 years, p = 0.4, and OS 40.6 versus 53.2 months, p = 0.3, respectively. One patient died of COVID-19. Nine patients had venous thromboembolisms (VTE), all of which occurred before the pandemic onset. Conclusions: There were no significant differences found in the mean age or OS of the MGUS patients who died before versus after the pandemic onset. An increase in VTE rates was not seen. Study results are limited by small patient numbers.

Patologías cutáneas asociadas a gammapatías monoclonales: ¿qué sabemos hasta ahora? / Dermatological manifestations of monoclonal gammopathies

Monoclonal gammopathies of uncertain significance (MGUS) correspond to pre-malignant hematological disorders characterized by the production of a monoclonal protein and infiltration of less than 10% of the bone marrow by plasma cells. Its importance lies in the risk of progression to malignant disorders and in the association with different renal, neurological and skin manifestations. There are pathophysiological mechanisms that support a causal relationship between monoclonal gammopathies (MGs) and different skin diseases, such as type I cryoglobulinemia (CG), primary systemic amyloidosis (PSA) or necrobiotic xanthogranuloma (NXG). However, there is a group of skin diseases associated with MGs whose pathogenesis has not been elucidated. In this context, the role of the dermatologist is crucial in the suspicion of different haematological disorders based on skin manifestations and in the multidisciplinary treatment of these patients. In this article, we carry out an exhaustive review of the literature published in this area and propose a screening algorithm for MGs in patients with specific skin diseases.

Clinical Analysis of Patients with MGUS, Primary Light Chain Amyloidosis, Multiple Myeloma or Multiple Myeloma with Concurrent Amyloidosis / 中国实验血液学杂志

OBJECTIVE@#To summarize and compare the clinical baseline characteristics of patients with monoclonal gammopathy of undetermined significance (MGUS), primary light chain amyloidosis (pAL), multiple myeloma (MM), or MM with concurrent amyloidosis, especially the differences in cytogenetic abnormalities.@*METHODS@#The clinical data of 15 cases of MGUS, 34 cases of pAL, 842 cases of MM and 23 cases of MM with concurrent amyloidosis were analyzed and compared retrospectively.@*RESULTS@#Cytogenetic statistics showed that the incidence of t (11; 14) in the four groups (MGUS vs pAL vs MM vs MM with concurrent amyloidosis) was 0%, 33.3%, 16.4%, and 15.8%, respectively (P=0.037); that of 13q deletion was 20.0%, 14.7%, 45.8% and 56.5%, respectively (P<0.001); gain of 1q21 was 50.0%, 12.5%, 47.4% and 40.9%, respectively (P=0.001). Proportion of pAL patients with 0, 1 and≥2 cytogenetic abnormalities (including 13q deletion, 17p deletion, 1q21 amplification and IgH translocation) accounted for 41.9%, 41.9% and 16.1%, respectively; while the proportion of the same category in MM was 17.6%, 27.3%, and 55.2% respectively; this ratio of MM with concurrent amyloidosis was more similar to MM. Subgroup analysis showed that genetic abnormalities (including 13q deletion, 17p deletion and 1q21 amplification) were comparable within t (11; 14) negative and positive groups. Compared with positive cases, t(11; 14) negative patients with MM or MGUS were more likely to have 13q deletions and multiple genetic abnormalities.@*CONCLUSION@#Clinical characteristics of pAL, especially cytogenetic abnormalities, are significantly different from MM with concurrent amyloidosis. It suggests that although the onset characteristics are similar, actually the two diseases belong to different disease subtypes which should be carefully predicted and identified.

Dysphagia as a Clinical Manifestation of Monoclonal Gammopathy of Undetermined Significance: A Case Report / 대한연하장애학회지

Swallowing can be affected by a variety of systemic diseases. The etiology of dysphagia in the geriatric population is usually overlooked due mainly to a presumed diagnosis of presbyphagia or difficulty in revealing the direct cause. On the other hand, dysphagia can be a meaningful clinical sign of premalignant systemic disease. A 78-year-old man, without any prior medical or family history, was admitted with the chief complaint of dysphagia with recent aspiration pneumonia. Instrumental swallowing tests revealed a severe degree of dysphagia due to decreased laryngopharyngeal sensation and weakness of the pharyngeal constrictor muscles. Extensive workup, including electromyography and laboratory tests, revealed severe sensorimotor peripheral polyneuropathy related to monoclonal gammopathy. Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant precursor of multiple myeloma, which is characterized by the proliferation of monoclonal proteins. These conditions are often associated with peripheral polyneuropathy, ataxia, and sometimes even muscle weakness. Although dysphagia can occur in other systemic disorders, such as vasculitis or paraneoplastic syndrome-related malignancies, there are few reports of dysphagia related to MGUS. The patient was followed up for three years. The MGUS showed no further progression, but the patient showed no improvement, indicating a protracted clinical course and poor prognosis when dysphagia is related to MGUS.

Incidence Analysis of Monoclonal Gammopathy of Undetermined Significance in People over 40 Years of Age / 中国实验血液学杂志

OBJECTIVE@#To study the distribution of monoclonal gammopathy of undetermined significance(MGUS) in different age, sex and ethnic people over 40 years old.@*METHODS@#Five hundred and ninety-six people(over 40 years old) examened in the Health Examination center of the First Affiliated Hospital of Xinjiang Medical University from July 2017 to September 2017 were selected. Among 596 people, male 310, female 286, Han people 488, and Uygur ethnic people 108. According to age, 596 people were divided into 3 groups, (40-59 years old group, 60-79 years old group, over 80 years old group). First, all samples were screened by capillary serum protein electrophoresis. If the suspected monoclonal bands were found in the electrophoretogram, and then the specific protein types were determined by serum immunofixation electrophoresis.@*RESULTS@#The total incidence of MGUS in 596 screened population was 4.027%. The incidence of MGUS in 40-59 years old group, 60-69 years old group and over 80 years old group were 1.762%, 2.929% and 10% respectively, and the differences among the groups were statistically significant(P<0.05). The incidence of MGUS in male (5.806%) was significantly higher than that in female (2.097%)(χ=5.177，P<0.05). Binary Logistic regression analysis showed that over 80 years old and male were independent risk factors for MGUS(P=0.001, OR=4.188, 95%CI: 1.814-9.673, P=0.048, OR=2.605, 95%CI: 1.009-6.725). The types of immunoglobulin in patients with MGUS were mostly IgG, IgG(66.7%) was significantly more than IgA (29.2%)(χ=21.375，P<0.05)，and there was no significant difference in the incidence of MGUS between people with in Kappa and Lambda.@*CONCLUSION@#The age increase and male may increase the incidence of MGUS, the IgG is the most common type of immunoglobcdin in pathogenesis of MGUS, so the early screening should be done.

Cadenas ligeras libres en suero en el diagnóstico de Gammapatías Monoclonales / Free light chains in serum in the diagnosis of Monoclonal Gammopathies

Las gammapatías monoclonales son un conjunto de enfermedades causadas por la proliferación clonal de células plasmáticas que secretan un mismo tipo de inmunoglobulina, conocido como inmunoglobulina clonal. El pronóstico de estas enfermedades hematológicas depende del diagnóstico temprano, en el Mieloma Múltiple se asocia con una enfermedad menos grave. Las pruebas de laboratorio desempeñan un papel importante en las distintas etapas del manejo clínico del paciente con gammapatía monoclonal. Estas incluyen pruebas tradicionales como la electroforesis de proteínas en suero, la inmunofijación, y actualmente se adiciona la determinación de cadenas ligeras libres en suero, una prueba complementaria importante porque permite evidenciar la presencia de gammapatías monoclonales de forma temprana por su alta sensibilidad, así como cuantificar las cadenas ligeras libres kappa/lambda y determinar la monoclonalidad a través del respectivo cociente, parámetro que desde el 2014 está integrado en los criterios diagnósticos de mieloma según la International Myeloma Working Group.Como metodología se realizó la búsqueda de material bibliográfico: se definieron las palabras clave utilizando los descriptores DeCS y MeSH, se inició la búsqueda de información en las bases de datos bibliográficas PubMed, ScienceDirect y se ejecutó una búsqueda en internet con buscador "google académico" con los mismos términos. Se seleccionaron aquellos artículos y páginas de internet que contaran con información de interés, que cumplieran los requisitos para incluirse dentro del artículo de revisión, se almacenaron en una base de datos y finalmente se realizó la lectura crítica de la información.

Monoclonal gammopathies are a group of diseases caused by the clonal proliferation of plasma cells that secrete the same type of immunoglobulin, known as clonal immunoglobulin. The prognosis of these hematological diseases depends on the early diagnosis, in Multiple Myeloma is associated with a less serious disease. Laboratory tests play an important role in the different stages of clinical management of patients with Monoclonal gammopathies, including traditional tests such as serum protein electrophoresis, immunofixation, and the determination of free light chains in serum it is considered an important complementary test since it allows initially to show the presence of Monoclonal gammopathies in an early way due to its high sensitivity, likewise it allows to quantify the kappa / lambda free light chains and to determine the monoclonality through the respective quotient, this is a parameter that since 2014 is integrated into the myeloma diagnostic criteria according to the International Myeloma Working Group.As a methodology, the search of bibliographic material was carried out: the keywords were defined using the DeCS and MeSH descriptors, the search for information in the PubMed, ScienceDirect bibliographic databases was started and an internet search was carried out with the "google academic" search engine with the same terms. Those articles and internet pages that had information of interest, that met the requirements to be included in the review article, were stored in a database and finally the critical reading of the information was selected

Crohn's disease and smoldering multiple myeloma: a case report and literature review

Crohn's disease (CD) is a chronic inflammatory bowel disease (IBD) that presents with abdominal pain, weight loss, and diarrhea. Although the etiology has not been fully elucidated, both environmental and genetic causes are known to be involved. In chronic inflammatory conditions such as IBD, B lymphocytes are chronically stimulated, and they induce monoclonal expansion of plasma cells, sometimes resulting in monoclonal gammopathy of undetermined significance. Immunomodulators that are commonly used to control inflammation, such as tumor necrosis factor-α (TNF-α) blockers could increase the possibility of hematologic malignancy. The pathogenesis of multiple myeloma in association with TNF-α inhibitor therapy is attributed to decreased apoptosis of plasma cell populations. Here, we describe a case of a 36-year-old male patient who was diagnosed with immunoglobulin A subtype smoldering multiple myeloma during the treatment for CD with infliximab and adalimumab. We report this case along with a review of the literature on cases of multiple myeloma that occurred in conjunction with CD.

Neutrophilic Leukemoid Reaction Associated with Malignancy Initially Suspected as Chronic Neutrophilic Leukemia

Although neutrophilia can manifest from various causes, it is important to be able to distinguish chronic neutrophilic leukemia (CNL) from neutrophilic leukemoid reactions (NLR). In this paper, we describe four cases of leukocytosis with neutrophilia, including one case of CNL with a T618I mutation in colony stimulating factor 3 receptor (CSF3R) and three cases of NLR associated with malignancy or sepsis, which were initially suspected as CNL. Of the three NLR cases, one was associated with ovarian cancer, one with monoclonal gammopathy of undetermined significance and one with multiple myeloma with sepsis. This study demonstrated that confirming the clonality of myeloid cells with CSF3R T618I could contribute to making an accurate differential diagnosis between CNL and NLR in patients with solid cancers or plasma cell neoplasms caused by paraneoplastic syndromes and/or infection.

Genetic abnormality in 101 cases of plasma cell dyscrasias by FISH technology and cytogenetic examination / 中南大学学报(医学版)

OBJECTIVE@#To investigate several abnormal genes by the fluorescence in situ hybridization (FISH) in multiple myeloma (MM), monoclonal gammopathy of undetermined significance (MGUS) and reactive plasmacytosis (RP), and to increase the diagnosis and differential diagnosis levels for these common plasma diseases. 
@*METHODS@#The clinical manifestations, image and laboratory tests and the FISH detection were retrospectively analyzed in 61 cases of newly diagnosed MM, 20 cases of MGUS and 20 cases of RP from August, 2012 to February, 2015 in the Xiangya Hospital of Central South University. 
@*RESULTS@#Fifty cases among 61 MM patients showed genetic abnormality by FISH technology. The total positive rate was 81.9%. Among them, 19 cases (31.1%) had 1q21 amplification, 18 cases (29.5%) lacked D13S319, 10 cases (16.4%) missed RB1, 10 cases (16.4%) had IGH translocation and 7 cases (11.4%) lacked p53 gene. The positive rate for two or more genes abnormal was 19.8% in 12 cases. However, in 20 cases of MGUS patients, the positive detection rate was 25%, including 4 cases (20%) of 1q21 augmentation and 2 cases (10%) of IGH translocation. There were not two or more abnormal genes in one case. While in RP cases, only 1 case of patients had D13S319 abnormal gene, and the positive rate was only 5%. There was significant difference (P<0.05) among the 3 groups. 
@*CONCLUSION@#The positive detection rate is 81.9% in MM patients by FISH, which is significantly higher than that in patients with MGUS or RP. FISH technology can detect a variety of abnormal genes in MM. It is useful for the differential diagnosis and prognosis for MM, MGUS and RP.

Expression of microRNA-221/222 in patients with monoclonal gammopathy of undetermined significance and multiple myeloma / 浙江大学学报·医学版

To detect the expression of miR-221/222 in serum and plasma cells in patients with monoclonal gammopathy of undetermined significance(MGUS) and multiple myeloma(MM), and to explore the possibility of miR-221/222 as biomarkers in the diagnosis and prognosis predicting of MGUS and MM.Bone marrow and serum samples from 14 patients with newly diagnosed MGUS, 81 patients with newly diagnosed or relapsed MM and 10 controls were collected from Sir Run Run Shaw Hospital of Zhejiang University and Tongde Hospital of Zhejiang Province during January 2013 and December 2015. The expressions of miR-221/222 in serum and in sorted CD138 positive plasma cells were detected by qRT-PCR, and the relative expression of miR-221/222 (Δct) was compared between the groups. Serum levels of miR-221 before and after treatment were compared in both remission group (=22) and refractory group (=13) in MM patients, and its correlation with serum level of β-MG was assessed using Pearson's correlation analysis.Serum levels of miR-221/222 in MGUS and MM groups were significantly higher than those in control group (all<0.01), while miR-221/222 levels in plasma cells were significantly lower in MGUS and MM groups than those in the control group (<0.05 or<0.01). No significant difference in miR-221/222 levels in serum and plasma cells was observed between MGUS group and MM group (all>0.05). There was no correlation between miR-221/222 levels in serum and plasma cells (=0.024 and -0.127, all>0.05), but miR-221 levels were correlated with miR-222 levels in both serum and plasma cells (=0.534 and 0.552, all<0.01). Receiver operating characteristic (ROC) curves showed that the areas under the curve (AUCs) of serum miR-221/222, plasma cell miR-221/222 in diagnosis of MGUS/MM were 0.968, 0.976, 0.801 and 0.727, respectively. There was no significant difference in serum level of miR-221 among MM patients with different paraprotein isotypes (>0.05), but serum level of miR-221 in patients with relapsed MM was higher than that in patients with newly diagnosed MM (<0.01). Compared with the patients with MGUS or MM stageⅠ and Ⅱ, patients with MM stage Ⅲ were of higher serum levels of miR-221 (<0.01). Serum level of miR-221 decreased after chemotherapy in the remission group (=51.5,<0.01), but such decrease was not observed in the refractory group (=67.5,>0.05). Serum level of β-MG was positively correlated with serum level of miR-221 (=0.524,<0.01).miR-221/222 in serum and plasma cells may be biomarkers for early diagnosis of MGUS, and are helpful for diagnosis and efficacy evaluation of MM.

Five-Year Follow-up Study of Monoclonal Gammopathy of Undetermined Significance in a Korean Elderly Urban Cohort / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: We previously reported the prevalence of monoclonal gammopathy of undetermined significance (MGUS) to be 3.3% among an elderly Korean urban cohort recruited during 2005-2006. Here, we report a 5-year follow-up study of the previously identified MGUS cohort. MATERIALS AND METHODS: The 680 participants from the initial cohort were followed-up for a median of 5 years. Sera were collected between 2010 and 2011. Two-step screening was performed with standard serum electrophoresis followed by immunofixation and determination of the serum concentration of monoclonal-protein (M-protein). RESULTS: Of the 680 participants (21 with MGUS), 348 (51%) agreed to participate in the follow-up study and 10 were found to have MGUS. Among the 21 MGUS patients initially identified, nine were followed-up, six had persistent M-protein, and one patient had progressed to multiple myeloma (progression rate, 1.0%/yr). The M-protein disappeared in the remaining two individuals. Among the 339 participants without MGUS who were followed-up, four developed an M-protein. There was no significant difference in survival with respect to the presence of MGUS (p=0.66). CONCLUSION: The 5-year follow-up data show that the natural clinical course of MGUS in Korea is similar to that in Western countries. MGUS was not associated with an increased risk of death over the 5-year study period.

Cryoglobulinemia with a Digital Gangrene from Monoclonal Gammopathy of Undetermined Significance

No abstract available.

Fibrillary glomerulonephritis combined with chronic inflammatory demyelinating polyneuropathy

A 58-yr-old man presented with leg edema and subacute weakness of his bilateral lower extremities. Urinary and serum immunoelectrophoresis revealed the presence of lambda-type Bence Jones proteins. He was ultimately diagnosed with monoclonal gammopathy of undetermined significance (MGUS). A renal biopsy specimen showed fibrillary glomerulonephritis (FGN), which was randomly arranged as 12-20 m nonbranching fibrils in the basement membranes. Immunofluorescence studies were negative for immunoglobulin (Ig)G, IgM, IgA, C3, and kappa light chains in the capillary walls and mesangial areas. A Congo red stain for amyloid was negative. Electromyography and nerve conduction velocity examinations results were compatible with the presence of demyelinating polyneuropathy. This case showed a rare combination of FGN, without Ig deposition, and MGUS combined with chronic inflammatory demyelinating polyneuropathy (CIDP).

Gammapatía monoclonal de significado incierto A propósito de un caso de nefropatía por depósito de cadenas ligeras lambda / Monoclonal gammopathy of undetermined significance Apropos of a case of lambda light chain deposition nephropathy

La nefropatía asociada a las gammapatías monoclonales es debida principalmente al depósito de cadenas ligeras. Las enfermedades renales paraproteinémicas son lesiones asociadas con depósito de inmunoglobulinas intactas o fragmentos de inmunoglobulinas (cadenas pesadas y cadenas ligeras). La enfermedad por depósito de cadenas ligeras es una condición rara, caracterizada por el depósito de cadenas ligeras monoclonales en muchos órganos y a nivel renal predominantemente en glomérulos y membranas basales tubulares. La enfermedad está frecuentemente asociada con desórdenes linfoproliferativos, y la mayoría de casos son causados por depósito de cadenas ligeras kappa. Aunque se presenta sobre todo en cuadros malignos, en ocasiones no se detecta patología hematológica y se denomina idiopática o "primaria". Suele manifestarse como una insuficiencia renal severa con proteinuria nefrótica, no tiene tratamiento claramente establecido y el pronóstico es malo. Se describen las características clínicas e histológicas del segundo caso informado en Colombia de nefropatía por depósito de cadenas ligeras diagnosticado en el contexto de una enfermedad renal paraproteinémica sin datos de malignidad. (Acta Med Colomb 2014; 39: 196-201).

Nephropathy associated with monoclonal gammopathies is mainly due to light chain deposition. The paraproteinemic kidney diseases are lesions associated with deposition of intact immunoglobulins or fragments of immunoglobulins (heavy and light chains). The disease due to deposition of light chains is a rare condition characterized by deposition of monoclonal light chains in many organs and as for the kidney, predominantly in glomeruli and tubular basement membranes. The disease is frequently associated with lymphoproliferative disorders and the majority of cases are caused by deposition of kappa light chains. Although presented primarily in clinical pictures of malignancy, sometimes no hematological pathology is detected and is called idiopathic or "primary". It usually manifests as severe renal failure with nephrotic proteinuria, has not a clearly established treatment and the prognosis is poor. The clinical and histological features of the second case reported in Colombia of a light chain deposition nephropathy diagnosed in the context of a kidney paraproteinemic disease without malignancy data, is presented. (Acta Med Colomb 2014; 39: 196-201).

Coexistence of AA and AL Cardiac Amyloidosis in a Patient with Ankylosing Spondylitis Accompanying Monoclonal Gammopathy of Undetermined Significance

Amyloidosis is a clinical disorder caused by extracellular deposition of proteinaceous insoluble fibrils in various tissues, resulting in organ compromise. Amyloid L (AL) amyloidosis is the most common type of systemic amyloidosis, which occurs in association with multiple myeloma or monoclonal gammopathy of undetermined significance (MGUS). Secondary amyloid A (AA) amyloidosis is a complication of chronic inflammatory conditions, such as rheumatoid arthritis or ankylosing spondylitis. We report a case of a 49-year-old manwith a 11-year history of ankylosing spondylitis, who was recently diagnosed with MGUS presented with cardiac amyloidosis of both the AA and AL types. We report this case along with a review of relevant literature.

Gammapatía monoclonal de significado incierto: factores de pronóstico, evolución y riesgo / Monoclonal gammopathy of undeterminaded significance: prognostic factor and risk of progression / Gamopatia monoclonal de significado indeterminado: fatores prognósticos: risco da evolução

La Gammapatía Monoclonal de Significado Incierto (GMSI) tiene una prevalencia que varía entre 1 y 3%, su frecuencia tiende a aumentar con la edad y aunque presentan evolución indolente y una sobrevida prolongada, un porcentaje de ellas desarrollará una enfermedad maligna. Con el objetivo de evaluar el valor pronóstico de diversos parámetros proteicos y hematológicos al momento del diagnóstico, se estudiaron mediante estudios proteicos completos en sangre y en orina, 407 pacientes con diagnóstico de GMSI que ingresaron a la institución en el período comprendido entre 1982 y 2008. La concentración del componente monoclonal (CM) (>1,5 g/dL) y el tipo inmunológico (No IgG), la disminución de las inmunoglobulinas no comprometidas (INC), el porcentaje de infiltración de células plasmáticas en médula ósea (>5%) y la mediana de las relaciones anormales de las cadenas livianas monoclonales libres, fueron los parámetros que marcaron riesgo de progresión a una enfermedad maligna. El estudio proteico completo de orina demostró una asociación entre el aumento en la concentración de proteínas de bajo peso molecular con valores de estimado de filtración glomerular menor de 60 mL/min/1,73 m2 y presencia de proteinuria de Bence Jones, independientemente del tipo de cadena liviana y de los niveles de proteínas totales. Debido a ello, la adición de dichos marcadores de daño tubular podría ofrecer una visión más profunda, siendo su aumento un posible indicador, en la profilaxis renal, de una severa lesión tubular futura. Finalmente, en pacientes con GMSI, los controles de laboratorio deberán ser ajustados en su periodicidad pero no en su contenido. La mayor información así obtenida será lo que permitirá una decisión médica más segura al momento de recomendar la frecuencia del seguimiento del paciente y la consiguiente detección temprana de una evolución maligna de la enfermedad.

Concomitant transformation of monoclonal gammopathy of undetermined significance to multiple myeloma and of essential thrombocythemia to acute biphenotypic leukemia 37 years after initial diagnosis

No abstract available.