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1.
Autops. Case Rep ; 11: e2021326, 2021. tab, graf
Article in English | LILACS | ID: biblio-1339247

ABSTRACT

Currently, there is growing evidence in the literature warning of misdiagnosis involving amyloidosis and chronic inflammatory demyelinating polyneuropathy (CIDP). Although inducing clinical manifestations outside the peripheral nervous system, light chain and transthyretin amyloidosis may initially present with peripheral neuropathy, which can be indistinguishable from CIDP, leading to a delay in the correct diagnosis. Besides, the precise identification of the amyloid subtype is often challenging. This case report exemplifies clinical and laboratory pitfalls in diagnosing amyloidosis and subtyping amyloid, exposing the patient to potentially harmful procedures.


Subject(s)
Humans , Male , Aged , Amyloidosis, Familial/complications , Paraproteinemias , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/complications , Diagnostic Errors , Immunoglobulin Light-chain Amyloidosis/complications
3.
Hematol., Transfus. Cell Ther. (Impr.) ; 42(3): 200-205, July-Sept. 2020.
Article in English | LILACS | ID: biblio-1134043

ABSTRACT

ABSTRACT Since the World has been facing the COVID-19 pandemic, special attention has been taken concerning cancer patients; related to their immunosuppression status, adding risk for more aggressive COVID-19 and mortality, but also concerns about the access and the quality of care in cancer therapy. The COVID-19 pandemic impacts the number of infected, its related mortality, as well as the care of cancer patients. Multiple myeloma patients are a particular group with several important aspects to be considered during pandemic times. In essence, they are immunosuppressed in different intensities during their treatment. Most of them are elderly and all of them require long-term therapy, with prolonged contact with the health care system, possibly including a stem cell transplant during the treatment. A panel of experts in multiple myeloma and infectious diseases discusses pieces of evidence and the lack of the same in the scenario of COVID-19 in myeloma patients, while also exposing what is expected for the next phases of the COVID-19 pandemic.


Subject(s)
Paraproteinemias , Stem Cell Transplantation , SARS-CoV-2 , COVID-19 , Multiple Myeloma/diagnosis , Multiple Myeloma/therapy
4.
Rev. cuba. reumatol ; 22(supl.1): e843, tab, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1280397

ABSTRACT

Las gammapatías monoclonales son un grupo amplio de enfermedades de células hematológicas con expresión clínica variable, con afectación sistémica o localizada. Muchos de estos trastornos simulan enfermedades reumáticas, y pueden presentarse previa- o posteriormente a la enfermedad de base, por lo cual dificultan su diagnóstico. El propósito de este estudio es comunicar los casos de cinco pacientes con manifestaciones clínicas de enfermedades reumáticas y diagnóstico final de enfermedades oncohematológicas. Se realizó un estudio descriptivo transversal mediante el análisis de historias clínicas de pacientes evaluados en el Servicio de Reumatología del Hospital José María Cullen de Santa Fe entre marzo del 2010 y junio del 2019. Se incluyó a cinco pacientes que fueron estudiados por sospecha de enfermedad reumatológica hasta llegar al diagnóstico final de gammapatía monoclonal. Cuatro pacientes presentaron mieloma múltiple manifestado como síndrome de Schnitzler; xantogranuloma del adulto y amiloidosis; aplastamientos vertebrales múltiples; falla renal aguda, respectivamente. El quinto paciente se presentó simulando una vasculitis sistémica con afectación multiorgánica y diagnóstico final de linfoma intravascular. Los pacientes fueron derivados al Servicio de Oncología y Hematología para su atención. A partir de la serie de casos analizados, se concluye que las manifestaciones reumáticas de las enfermedades oncohematológicas se deben tener presentes en el accionar diario para evitar la demora diagnóstica y los tratamientos innecesarios(AU)


Monoclonal gammapathies are a broad group of diseases from hematopoietic cells with variable clinical features and systemic or limited involvement. These entities could begin as a rheumatic disease, even previously to the diagnosis of MG. To describe five patients with rheumatic manifestations that lately were diagnosed as monoclonal gammapathies. We describe the more relevant features of five patients assisted in our rheumatology center. Four patients were diagnosed with multiple myeloma that begins as: 1) Schnitzler's syndrome, 2) Adult-onset xanthogranuloma and amyloidosis, 3) multiple vertebral fracture, 4) acute kidney failure. The 5th patient has a vasculitis-like syndrome due to an intravascular lymphoma. The rheumatic-like syndromes are infrequent but we should take into account this diagnosis in our clinical practice for rapid diagnostic and correct treatment(AU)


Subject(s)
Humans , Rheumatic Diseases , Hematology , Medical Oncology , Paraproteinemias/diagnosis , Epidemiology, Descriptive , Cross-Sectional Studies
6.
Article in English | WPRIM | ID: wpr-785401

ABSTRACT

BACKGROUND: Waldenström macroglobulinemia (WM) is a subset of lymphoplasmacytic lymphoma (LPL) with bone marrow (BM) involvement and an IgM monoclonal gammopathy of any level. We aimed to identify the clinical, laboratory, and BM findings of patients with WM and to evaluate the usefulness of CD154 for the diagnosis and prognosis of WM.METHODS: We reviewed the medical records and BM studies and/or flow cytometric immunotyping of 31 patients with untreated WM. Semiquantitative immunohistochemistry (CD20, CD138, tryptase, and CD154) of BM was performed.RESULTS: Only six patients presented with symptoms of hyperviscosity syndrome. Eleven patients had solid cancer and/or another hematologic malignancy. Mast cells (MC) increased in all samples, with some in close contact with tumor cells. Tryptase-positive MC (17.1/ high-power fields [HPF], 1.2–72.0/HPF) and CD154-positive MC (8.6/HPF, 0.1–31.1/HPF) were observed. The high CD154-positive MC (≥8.6/HPF) group showed a lower overall five-year survival rate than the low CD154-positive MC (<8.6/HPF) group (71.9% vs. 100.0%; P=0.012). Flow cytometric immunophenotyping of BM aspirates showed increased B lymphocytes and plasma cells with a normal phenotype (CD138⁺/CD38⁺/CD19⁺/CD45⁺/CD56⁻).CONCLUSIONS: Approximately one third of WM patients showed other malignancies and all patients had increased MC. Immunohistochemistry and flow cytometric immunophenotyping are useful for diagnosing WM, and increased CD154-positive MC can indicate poor prognosis.


Subject(s)
B-Lymphocytes , Bone Marrow , Diagnosis , Hematologic Neoplasms , Humans , Immunoglobulin M , Immunohistochemistry , Immunophenotyping , Lymphoma , Mast Cells , Medical Records , Paraproteinemias , Phenotype , Plasma Cells , Prognosis , Survival Rate , Tryptases , Waldenstrom Macroglobulinemia
7.
Article in Chinese | WPRIM | ID: wpr-827175

ABSTRACT

OBJECTIVE@#To investigated the clinical and pathological characteristics of related-renal damage in patients with POEMS syndrome.@*METHODS@#Five patients diagnosed as POEMS syndrome in our hospital were selected. Their clinical manifestation, pathological characteristics of kidney and laboratory examination were analyzed retrospectively. Among the 5 patients, three males and two females with a median age of 50 years old. The mean interval before diagnosis was 13.0±7.2 months.@*RESULTS@#All the patients showed neuropathy, endocrinopathy, monoclonal plasma cell-proliferative disorder, skin changes and extravascular volume overload, in which 4 patients showed organomegaly. Proteinuria was found in 5 patients, and microhematuria was found in 4 patients. Moreover, 4 patients showed an elevated blood urea, while 2 patients showed creatinine elevation. 1 patient at chronic kidney disease (CKD)-G1 stage, 2 patients at CKD-G2 stage, and 1 patient at CKD-G3b stage, moreover, 1 patient at CKD-G5 stage. Endothelial injury and mesangial lesion were the main characteristics of renal pathology. 3 patients were pathologically diagnosed as thrombotic microangiopathy kidney damage, while 2 patients as light chain amyloidosis.@*CONCLUSION@#POEMS syndrome is a multi-systemic disease with complex clinical manifestations. 5 patients had different degrees of renal insufficiency. Endothelial injury and mesangial lesion are the main features of renal pathology.


Subject(s)
Female , Humans , Kidney , Male , Middle Aged , POEMS Syndrome , Paraproteinemias , Renal Insufficiency , Retrospective Studies
8.
Rev. cuba. hematol. inmunol. hemoter ; 35(4): e1092, oct.-dic. 2019. tab, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1093296

ABSTRACT

Introducción: Los avances en el manejo del mieloma múltiple (MM) durante los últimos años incluyen la incorporación del trasplante de progenitores hematopoyéticos autólogo (TPHa) a la estrategia de tratamiento de estos pacientes. Objetivo: Dar a conocer los primeros resultados en el hospital Hermanos Ameijeiras (HHA) con la aplicación del TPHa en pacientes con gammapatías monoclonales (GM), empleando las altas dosis de melfalán (AD-Mel) como tratamiento acondicionante (TA) y su impacto en la sobrevida global (SG). Métodos: Se hizo un estudio retrospectivo de todos los pacientes con GM sometidos a TPHa en el Servicio de Hematología del HHA en el período comprendido entre 2009 y 2018. La muestra final comprendió 14 casos. Resultados: La edad promedio fue de 53,5 años; la mayoría tenía como diagnóstico MM (85,7 por ciento) y todos ellos debutaron en estadio III de Durie-Salmon; como TA el 64,2 por ciento recibió AD-mel, en dosis de 200 mg/m2. La recuperación de las cifras de neutrófilos y plaquetas ocurrieron como promedio a los 11,4 y 12 días, respectivamente. La mortalidad relacionada con el trasplante (MRT) al día +30 fue del 7,1 por ciento. La probabilidad de SG a los 2 años fue superior al 90 por ciento y a los 5 años del 68 por ciento. Conclusiones: Se comprobó que la realización del TPHa con el empleo de AD-Mel como TA en pacientes con GM es un proceder realizable en nuestro país con una MRT relativamente baja. Se logró demostrar que la inclusión del TPH en el tratamiento mejora considerablemente las expectativas de sobrevida de estos pacientes(AU)


Introduction: The recent advances in the management of multiple myeloma (MM) during the last years have included the autologous hematopoietic stem cell transplantation (auto-HSCT) to the treatment strategy of these patients. Objective: To present the first results in the Hermanos Ameijeiras hospital (HAH) with the application of auto-HSCT in patients with monoclonal gammopathies (MG) using high doses of melphalan (HD-Mel) as conditioning regimen (CR) and its impacton overall survival (OS). Methods: A retrospective study of all patients with MG who underwent auto-HSCT in the Hematology Service of the HAH in the period between 2009 and 2018 wasmade. The final sample comprised 14 cases. Results: The average age was 53.5 years; the majority had diagnosis of MM (85.7percent) and all of them were diagnosed in stage III of Durie-Salmon; as CR 64.2 percent received HD-mel, at 200 mg/m2. The recovery of neutrophil and platelet counts occurred on average at 11.4 and 12 days respectively. Transplant related mortality (TRM) at day +30 was 7.1 percent. The probability of OS at 2 years was higher than 90 percent and at 5 years of 68 percent. Conclusions: It was verified that the performance of auto-HSCT with the use of HD-Mel as CR in patients with MG is a feasible procedure in our country with a relatively low TRM. It was possible to demonstrate that the inclusion of auto-HSCT in the treatment considerably improves the survival expectations of these patients(AU)


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Paraproteinemias/therapy , Hematopoietic Stem Cell Transplantation/methods , Melphalan/therapeutic use , Survival Analysis , Retrospective Studies , Multiple Myeloma/therapy
9.
Rev. cuba. hematol. inmunol. hemoter ; 35(3): e1067, jul.-set. 2019.
Article in Spanish | LILACS, CUMED | ID: biblio-1093277

ABSTRACT

Introducción: El mieloma múltiple (MM) es una enfermedad que va precedida por una fase previa conocida como gammapatía monoclonal de significado incierto (GMSI); en esta última existen varias anormalidades citogenéticas, que permiten la progresión a MM, entre estas encontramos reordenamientos primarios del gen de la cadena pesada de la inmunoglobina (IGH), además de células hiperdiploides. Desarrollo: Las alteraciones cromosómicas en el MM se pueden clasificar en dos grupos principales: las que involucran las translocaciones del locus IGH ubicado en el cromosoma 14q32 y cuyos principales reordenamientos se dan entre las regiones cromosómicas 11q13, 16q23, 4p16.3, 6p21 y, un segundo grupo caracterizado por los desequilibrios genómicos. Los pacientes con translocaciones de la IGH, muestran un pronóstico diferente en dependencia del tipo de reordenamiento cromosómico. La t(4;14)(p16;q32) y t(14;16)(q32;q23) se asocian a un mal pronóstico, mientras que los pacientes con t(11;14) (q13;q32) tiene un buen pronóstico de la enfermedad en ausencia de otras anormalidades genéticas. En el grupo con desequilibrio genómico se encuentran deleciones, amplificaciones, y células con números anormales de cromosomas (hiperdiploidas y no hiperdiploides); casi siempre asociadas a mal pronóstico ya que muchas de estas alteraciones involucran perdida de material genómico relacionado con el control de ciclo celular y progresión de la enfermedad, como son las deleciones de los cromosomas 1,13 y 17. Los pacientes con trisomías de los cromosomas impares 1, 3, 5, 7, 9, 11, 15, 19,21 suelen tener un mejor pronóstico y una tasa mayor de sobrevivencia(AU)


Introduction: Multiple myeloma (MM) is a disease that is preceded by a previous phase known as monoclonal gammopathy of uncertain significance (MGUS); in this latter there are several cytogenetic abnormalities, which allow the progression to MM, among these we find primary rearrangements of the heavy chain gene of the immunoglobin (IGH), in addition to hyperdiploid cells. Development: Chromosomal alterations in MM can be classified into two main groups, those involving the translocations of the IGH locus located on chromosome 14q32 and whose main rearrangements occur between the chromosomal regions 11q13, 16q23, 4p16.3, 6p21, and a second group which is characterized by genomic imbalances. Patients with translocations of the IGH, show a different prognosis depending on the type of chromosomal rearrangement, the t(4; 14)(p16; q32) and t(14; 16)(q32; q23) are associated with a poor prognosis while patients with t(11; 14)(q13; q32) have a good prognosis of the disease in the absence of other genetic abnormalities. Within the genomic imbalances we find deletions, amplifications, and cells with abnormal numbers of chromosomes (hyperdiploids and not hyperdiploid), these almost always associated with poor prognosis since many of these alterations involve loss of genomic material related to cell cycle control and progression of the disease, such as deletions of chromosomes 1,13 and 17. Patients with trisomies of odd chromosomes 1, 3, 5, 7, 9, 11, 15, 19,21 usually have a better prognosis and a higher survival rate(AU)


Subject(s)
Humans , Male , Female , Prognosis , Multiple Myeloma/genetics , Multiple Myeloma/epidemiology , Paraproteinemias/genetics , Chromosome Deletion , Disease Progression , Cytogenetic Analysis/methods , Bortezomib/therapeutic use
10.
Rev. méd. Chile ; 147(8): 1036-1041, ago. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1058640

ABSTRACT

Hematological neoplasms are tumors of cells in different states of maturation and differentiation. Since monoclonal gammopathies (MG) refer to B mature lymphocyte neoplasms, lymphogenesis should be well known. We must keep in mind that the last stage of maturation of these lymphocytes is the plasma cell. This is how a MG could appear in the context of a plasma cell neoplasm, such as multiple myeloma or amyloidosis, but also in relation to a lymphoma. A monoclonal peak is produced by mature B lymphocytes or plasma cells that secrete a monoclonal protein (Immunoglobulin), and represents a MG. But it must be emphasized that, in the correct clinical context, a hypogammaglobulinemia can represent a MG as well. Another important point is the understanding and interpretation of requested tests, such as protein electrophoresis (PEP), immunofixation (IFx) or serum free light chains (sFLC). The current MG screening panel includes these three studies (PEF, IFx, sFLC), although a simpler panel measuring PEF and sFLC has also been proposed, but not yet formally validated. Therefore, screening done only with PEP is insufficient.


Subject(s)
Humans , Paraproteinemias/blood , Paraproteins/analysis , Neoplasms, Plasma Cell/blood , Paraproteinemias/diagnosis , Blood Protein Electrophoresis/methods , B-Lymphocytes/metabolism , Neoplasms, Plasma Cell/diagnosis
11.
In. CASMU. Investigación clínica: desarrollo e innovación, 2019. Montevideo, Ideas Uruguay, 2019. p.206-206.
Monography in Spanish | LILACS, BNUY, UY-BNMED | ID: biblio-1359605
12.
Braz. j. med. biol. res ; 52(7): e8222, 2019. graf
Article in English | LILACS | ID: biblio-1011591

ABSTRACT

Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.


Subject(s)
Humans , Male , Middle Aged , Paraproteinemias/etiology , Purpura, Schoenlein-Henoch/complications , Nephritis/complications , Paraproteinemias/pathology , Paraproteinemias/drug therapy , Purpura, Schoenlein-Henoch/pathology , Purpura, Schoenlein-Henoch/drug therapy , Glucocorticoids/administration & dosage , Immunosuppressive Agents/administration & dosage , Nephritis/pathology , Nephritis/drug therapy
13.
Rev. méd. Chile ; 147(1): 18-23, 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-991368

ABSTRACT

Background: Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). Aim: To report the features of patients with pPCL. Material and Methods: Review of databases of the Hematology Department and the Hematology laboratory. Results: Of 178 patients with monoclonal gammopathies, five (2.8%) patients aged 33 to 64 years (three females) had a pPCL. The mean hemoglobin was 7.3 g/dL, the mean white blood cell count was 52,500/mm3, with 58% plasma cells, and the mean platelet count was 83,600/mm3. The mean bone marrow infiltration was 89%, LDH was 2,003 IU/L, serum calcium was 13 mg/dL, and creatinine 1.5 mg/dL. Two patients had bone lesions. Three were IgG, one IgA lambda and one lambda light chain. CD20 was positive in one, CD56 was negative in all and CD117 was negative in 3 cases. By conventional cytogenetic analysis, two had a complex karyotype. By Fluorescence in situ Hybridization, one was positive for TP53 and another for t (11; 14). One patient did not receive any treatment, three patients received VTD PACE and one CTD. None underwent transplant. Three patients are alive. The mean survival was 14 months. Conclusions: These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Leukemia, Plasma Cell/genetics , Leukemia, Plasma Cell/epidemiology , Paraproteinemias/genetics , Paraproteinemias/pathology , Paraproteinemias/epidemiology , Blood Cell Count , Leukemia, Plasma Cell/pathology , Leukemia, Plasma Cell/therapy , Survival Analysis , Chile/epidemiology , Calcium/blood , Retrospective Studies , Treatment Outcome , In Situ Hybridization, Fluorescence , Creatinine/blood , Cytogenetic Analysis , Flow Cytometry/methods
14.
Article in Spanish | LILACS | ID: biblio-1087377

ABSTRACT

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


Subject(s)
Humans , Paraproteinemias , Smoldering Multiple Myeloma , Monoclonal Gammopathy of Undetermined Significance
16.
Article in English | WPRIM | ID: wpr-762438

ABSTRACT

POEMS syndrome is a rare paraneoplastic syndrome, which includes polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes due to plasma cell (PC) neoplasm. Diagnosis of this disease is challenging because of its rarity and complex clinical manifestations. We attempted to identify the key clinical features and characteristic bone marrow (BM) findings of POEMS syndrome, by reviewing the medical records and BM analyses of 24 Korean patients. Frequent clinical manifestations included polyneuropathy (100%), monoclonal gammopathy (100%), organomegaly (92%), extravascular volume overload (79%), and endocrinopathy (63%). The BM analyses revealed mild PC hyperplasia (median PCs: 5.5%) and frequent megakaryocytic hyperplasia (88%), megakaryocyte clusters (88%), and hyperlobation (100%). Flow cytometry of BM aspirates using CD138/CD38/CD45/CD19/CD56 showed normal (67%, 4/6) or neoplastic PC immunophenotypes (33%, 2/6). A diagnosis of POEMS syndrome must be considered when a patient suspected of having PC dyscrasia shows the above clinical presentation and BM findings.


Subject(s)
Bone Marrow , Diagnosis , Flow Cytometry , Humans , Hyperplasia , Medical Records , Megakaryocytes , Paraneoplastic Syndromes , Paraproteinemias , Plasma Cells , POEMS Syndrome , Polyneuropathies , Skin
17.
Article in Korean | WPRIM | ID: wpr-759693

ABSTRACT

Diffuse plane xanthoma (DPX) presents with symmetric yellow-orange plaques primarily on the neck, upper trunk, flexural folds, and the periorbital region. Based on serum lipid and lipoprotein levels, these xanthomas are classified as normolipemic or hyperlipoproteinemic DPX. Diffuse normolipemic plane xanthoma (DNPX) is a rare condition that is not well studied yet. It is associated with reticulo-endothelial diseases, particularly multiple myeloma and monoclonal gammopathy of unknown significance (MGUS). A 62-year-old woman developed yellowish hyperpigmented papules and diffuse patches in the medial canthal area of her neck. Based on a skin biopsy and laboratory analyses, she was diagnosed with DNPX associated with multiple myeloma. This diagnosis demonstrates that dermatological lesions should be carefully assessed as they may be the first manifestation of an underlying hematological disease. We report herein a rare case of diffuse plane xanthoma associated with multiple myeloma and review the relevant literature.


Subject(s)
Biopsy , Diagnosis , Female , Hematologic Diseases , Humans , Lipoproteins , Middle Aged , Multiple Myeloma , Neck , Paraproteinemias , Skin , Xanthomatosis
18.
Article in English | WPRIM | ID: wpr-766401

ABSTRACT

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.


Subject(s)
Aged , Ataxia , Deglutition , Deglutition Disorders , Diagnosis , Electromyography , Hand , Humans , Monoclonal Gammopathy of Undetermined Significance , Multiple Myeloma , Muscle Weakness , Muscles , Paraproteinemias , Pneumonia, Aspiration , Polyneuropathies , Prognosis , Sensation , Vasculitis
19.
Laboratory Medicine Online ; : 263-268, 2019.
Article in Korean | WPRIM | ID: wpr-760506

ABSTRACT

Lymphoplasmacytic lymphoma (LPL) is a low-grade B-cell neoplasm, composed of small B lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving bone marrow and sometimes lymph nodes or spleen. LPL with bone marrow involvement and an IgM monoclonal gammopathy of any concentration is designated as Waldenström macroglobulinemia (WM). LPL associated with non-IgM monoclonal gammopathy or biclonal gammopathy is rarely observed. LPL diagnosis was based on clinical, morphological, and immunophenotypic findings. Recently, the test for L265P mutation of the myeloid differentiation factor 88 (MYD88) gene has been helpful in the diagnosis of LPL. Here, we reported the first case of LPL/WM with IgM-κ/IgA-λ biclonal gammopathy in Korea.


Subject(s)
B-Lymphocytes , Bone Marrow , Diagnosis , Immunoglobulin M , Korea , Lymph Nodes , Lymphocytes , Lymphoma , Multiple Myeloma , Myeloid Differentiation Factor 88 , Paraproteinemias , Plasma Cells , Spleen , Waldenstrom Macroglobulinemia
20.
Article in Chinese | WPRIM | ID: wpr-771909

ABSTRACT

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.


Subject(s)
Adult , Aged , Aged, 80 and over , Female , Humans , Immunoglobulins , Incidence , Male , Middle Aged , Monoclonal Gammopathy of Undetermined Significance , Paraproteinemias , Risk Factors
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