Diagnostic and prognostic value of bone marrow plasma cell morphology in patients with plasma cell myeloma / 中华检验医学杂志

Objective:To investigate the value of bone marrow plasma cell morphology in the diagnosis and prognosis of plasma cell myeloma (PCM).Method:Observational study.Collect the bone marrow morphology image reports and corresponding monoclonal protein (M protein) identification results of 1071 patients [629 males and 442 females, Median age 62 (29, 93) years] diagnosed with PCM in the outpatient and inpatient departments of Beijing Chaoyang Hospital affiliated to Capital Medical University from January 1, 2017 to February 28, 2022. Combined with Durie‐Salmon(DS) and International Staging System (ISS) of 427 patients diagnosed with PCM and overall survival time (OS) of 436, summarize the relevant plasma cell morphological characteristics. Statistical methods include chi-square test, Kruskal-Walls test, Spearman correlation analysis and Kaplan-Meier survival analysis.Result:The bone marrow morphology reports showed that the typical morphological features of peripheral blood in 573 patients with PCM included plasma cells (40.84%), immature granulocytes (30.89%), rouleaux formation in erythrocytes (68.94%) and nucleated red blood cells (8.55%). The types of bone marrow plasma cells in 1 071 patients diagnosed with PCM included 372 (34.73%) plasmablasts, 674 (62.93%)immature plasma cells, and 25 (2.34%) mature plasma cells. There is a significant positive correlation between the number of bone marrow plasma cells (proportion of nuclear cells) and the concentration of IgG and IgA type, from M protein identification( r=0.55, r=0.60, P<0.01). The proportions of M protein types in 1 071 patients with PCM from high to low were IgG (45.75%), IgA (23.53%), light chain (19.61%), IgD (4.76%), non-secretory (4.3%), biclonal (1.78%), IgE (0.19%), IgM (0.08%). The typical characteristics of the bone marrow plasma cells in various M protein types included clustered distribution, different cell body sizes, inclusions in the cytoplasm, binuclear, polynuclear, and abnormal nuclear. The proportion of plasmablasts in DSⅢ stage was 44.81% (164/366), higher than 21.57% (11/51) in DSⅡstage, and the difference was statistically significant(χ 2=10.2, P<0.05). There was a significant positive correlation between the number of bone marrow plasma cells and DS and ISS stages( r=0. 0.23, r=0.30, P<0.01). The median OS of the PCM patients in the plasmablasts group was significantly shorter than that in the immature plasma cells group [56.0 (23.0, 101.8) months vs 75.9(31.6, 121.5) months, HR=1.42,95% CI 1.05-1.91, P=0.02]. The median OS of the PCM patients in the group of tumor plasma cells burden≥37.5% was shorter than that of the tumor plasma cells burden<37.5% [75.9 (21.4, 122.6)months vs 81.3 (36.6, 108) months, HR=1.54,95% CI 1.14-2.07, P<0.05]. Conclusion:The morphology and tumor burden of bone marrow plasma cells provide an important basis for the diagnosis of PCM and can be used as a prognostic indicator for patients with PCM.

Mieloma múltiple: la radiografía panorámica como un primer hallazgo de diagnóstico / Multiple myeloma: ray wide as a first discovery of diagnosis

Mieloma múltiple (MM), también conocido como Mieloma de células plasmáticas, es una proliferación tumoral de plasmocitos que infiltran la médula hematopoyética, de causa desconocida. Recursos de imagen son ampliamente utilizados en clínica estomatológica y los profesionales de esta área puede ser el primero en ser solicitado, por lo tanto, es importante la detección de la afectación que puede tener carácter sistémico en la preservación de la salud del paciente. Bajo estas condiciones, la literatura tiene pruebas de la ocurrencia del mieloma múltiple. En este reporte, una paciente mujer, de raza negra, de 53 años de edad fue remitida al Departamento de Estomatología por un odontólogo, por la presentación de las lesiones radiolúcidas en la mandíbula. Los hallazgos radiográficos panorámicos incluyen múltiples lesiones mal definidas radiolúcidas y la gammagrafía ósea mostró múltiples áreas de captación del radiotrazador en la mandíbula, la pelvis, la columna vertebral, el cráneo, la clavícula y las costillas. La paciente fue remitida a un comité de tumores multidisciplinar, y ha tolerado el tratamiento y su seguimiento desde el año 2009. Debido al comportamiento de esta enfermedad, el cuidado clínico y la evaluación radiológica deben llevarse a cabo, proporcionando información precisa y guías de tratamiento de los pacientes.

Multiple myeloma (MM), also called plasma cell myeloma, is a monoclonal neoplasic proliferation of plasma cells of bone marrow derivation with an unknown cause. Imaging resources are widely used in clinical stomatology and the professionals of this area may be the first to be requested, thus, it is important the detection of involvement that may have systemic character in preserving the patient's health. Under these conditions, the literature has proof the occurrence of the multiple myeloma. In this report the 53-year-old melanoderm woman was referred to the Stomatology Department by a dentist on presentation of radiolucent lesions in the mandible. At the panoramic radiographic findings included multiple ill-defined radiolucent lesions and bone scintigraphy showed multiple areas of radiotracer uptake in the jaw, pelvis, spine, skull, clavicle, and ribs. The patient was referred to a multidisciplinary tumor board, and she has been tolerating her treatment well and following up since 2009. Due to the behavior of this disease, careful clinical and radiological evaluation must be carried out, which provides accurate information and guides treatment of patients.

Mandible plasma cell myeloma:A case report / 实用口腔医学杂志

A case of mandible plasma cell myeloma is reported.Plasma cell myeloma is a monoclonal neoplastic proliferation of plasma cells of bone-marrow derivation,usually multi-centric but jaw bones are rarely involved.The treatment requires precise pathological diagno-sis.The prognosis of solitary jaw bone lesions can be good after treatment.

Bleeding Tendency of a Light Chain (AL) Amyloidosis Patient Accompanied by Asymptomatic Plasma Cell Myeloma

We present a case of abrupt-onset hemorrhagic tendency in a patient with amyloidosis who also had asymptomatic plasma cell myeloma. The patient was a 66-yr-old man with no previous history of hemorrhagic tendency and no family history of hemorrhagic disease. On examination, the prothrombin time and activated partial thromboplastin time were found to be prolonged and were not corrected even after a mixing test; moreover, the levels of coagulation factors I, II, V, VII, and X were almost normal. We therefore considered the presence of a nonspecific coagulation inhibitor. Although the von Willebrand factor (vWF) activity and vWF antigen level were normal due to sampling following transfusion, the increased closure time on PFA-100 (Siemens) analysis and the absence of ristocetin-induced platelet aggregation suggested the presence of acquired von Willebrand syndrome (vWS). After chemotherapy, the patient showed alleviation in the bleeding symptoms. Therefore, testing for acquired vWS should be considered when a patient has a history of recent bleeding with underlying amyloidosis.

Morfología dual de células de mieloma: inusual presentación de un caso / Unusual presentation of plasma cell myeloma

Una mujer de 41 años consultó por dolor facial. En una resonancia magnética nuclear se observó una masa en el ápex del peñasco derecho. La biopsia mostró una infiltración difusa por células grandes atípicas con morfología plasmablástica, positivas para CD138, BCL6, CD56 y p53, con expresión monoclonal de cadena liviana kappa y factor de proliferación del 80%, planteando el diagnóstico diferencial entre linfoma plasmablástico versus plasmocitoma plasmablástico. Un mapeo óseo evidenció múltiples lesiones osteolíticas en cráneo; el proteinograma reveló hipogamaglobulinemia y la inmunofijación en suero y orina fueron negativas. Se realizó biopsia de médula ósea donde se observó infiltración en un 30% del cilindro óseo por células plasmáticas maduras monoclonales para kappa, con expresión focal de p53 y negativas para CD56. Estos hallazgos confirmaron el diagnóstico de mieloma múltiple. Este caso pone de manifiesto la existencia de un espectro morfológico de las neoplasias de células plasmáticas, mostrando una evolución clonal continua con una plasticidad adquirida para desdiferenciarse, volverse inmaduras e infiltrar tejidos extramedulares, posiblemente debido a acumulación de alteraciones moleculares. Por lo tanto, se evidencia la dificultad del diagnóstico diferencial histopatológico entre linfoma plasmablástico y transformación plasmablástica de mieloma múltiple, debido a sus perfiles inmunohistoquímicos casi idénticos.

A 41 year-old woman consulted because of facial pain. A magnetic resonance imaging showed a mass in the right petrous apex. A biopsy revealed a diffuse proliferation of large atypical cells with plasmablastic appearance, positive for CD138, BCL6, CD56 and p53. The proliferation factor was 80%. Monoclonal kappa light chain expression was observed. Because the unusual clinicopathological features the patient was studied to rule out systemic plasma cell myeloma. Bone scan disclosed multiple cranium osteolytic lesions; proteinogram showed hypogammaglobulinemia and immunofixation in serum and urine were negative. Afterwards, bone marrow biopsy was performed and it presented a 30% infiltration of the bone cylinder by mature plasma cells. These were monoclonal for kappa light chain with focal expression of p53 and without expression of CD56. These findings suggested the diagnosis of multiple myeloma. This case proposes a morphological spectrum of plasma cell neoplasms, showing a continuous clonal evolution of tumor cells, with an acquired plasticity of dedifferentiate, become immature and infiltrate extramedullary tissues, a fact possibly determined by accumulation of multiple genetic alterations. These findings confirm the difficulty of the differential diagnosis from histopathology study between plasmablastic lymphoma and plasmablastic transformation of plasma cell myeloma because of the nearly identical immunohistochemical profiles.

A Case of Intracranial Involvement in Plasma Cell Myeloma / 영남의대학술지

Plasma cell myelomas generally manifest as bone or soft-tissue tumors with variable mass effects, pain, and infiltrative behavior. Extramedullary involvement occurs most commonly in the spleen, liver, lymph nodes, and kidneys, but intracranial involvement in plasma cell myeloma is a rare extramedullary manifestation. These authors recently encountered a case of intracranial involvement of plasma cell myeloma. A 69-year-old man was hospitalized for headache and mental changes. Brain CT showed subdural hemorrhage caused by plasma cell myeloma. Plasma cell myeloma with intracranial involvement has poor prognosis, and the patient in this case died from acute complications, such as subdural hemorrhage. Based on this case report, it is suggested that more effective treatment regimens of plasma cell myeloma with intracranial involvement be developed. Moreover, a screening method and decision on the appropriate time for intracranial involvement are needed for plasma cell myeloma patients.

Immunoglobulin Isotype Switching in a Plasma Cell Myeloma Patient Treated with High-dose Chemotherapy and Hematopoietic Stem Cell Transplantation

A malignant plasma cell clone usually produces a single abnormal monoclonal protein with a constant isotype. However, switching of paraprotein isotype has been reported to be a transient phenomenon associated with the recovery of B-cell function, and, in some cases, the switching might be misinterpreted as relapse. In August 2008, we encountered a case of a 59-year-old man with proteinuria and high IgG level (5.6 g/dL), kappa free light chain level of 2,660 mg/L, reversed A/G ratio (0.51), and multiple osteolytic lesions. Plasma cells, which accounted for 57% of all the nucleated cells, in bone marrow aspirates were positive for kappa immunostaining. Serum protein electrophoresis showed one M-spike, concentration of 4.87 g/dL in the beta region. Immunofixation electrophoresis revealed the peak as an IgG-kappa monoclonal protein; therefore, a diagnosis of plasma cell myeloma was made. Complete remission was achieved after chemotherapy, and autologous peripheral stem cell collection was performed. In March 2009, the patient underwent high-dose chemotherapy and autologous peripheral stem cell transplantation support. After 2 months, serum protein electrophoresis showed 2 M-spikes in the gamma region with positive IgM-lambda, IgG-lambda, and IgG-kappa, and these bands persisted. The electrophoretic mobility of the IgG-kappa protein was different from that of the original disease protein, and bone marrow results were the same as the previous ones. Although immunoglobulin isotype switch is known to have a benign course, it always requires careful monitoring because, in rare cases, true clonal switching may occur.

Variant Burkitt-type translocation (8;22)(q24;q11) in plasma cell myeloma

Variant Burkitt-type translocation, t(8;22)(q24;q11), is very rare in plasma cell myeloma. We report a 51-year-old male patient with plasma cell myeloma, who showed t(8;22) (q24;q11). He suffered from pelvic pain for two months, and showed IgG, lambda type of monoclonal gammopathy (5.14 g/dL; 49.9% of protein). His bone marrow examination showed increased plasma cells (66.9% of all nucleated cells). Plasma cells (74.9% of all nucleated cells) and monoclonal spike (3.38 g/dL; 42.2%) persisted after three cycles of thalidomide and dexamethasone. Cytogenetic analysis showed complex chromosomal abnormalities: 44,XY,-1,t(2;5)(q33;q13),add(8)(q24.1),t(8;22)(q24.1;q11.2),add(10) (p15), der(11)t(1;11)(q21;p11.2),del(12)(p11.2p13),-13,-14,add(14)(q32),der(15)t(1;15)(p2 2;p11.2),-16,add(17)(q11.2),+21,+1-3mar[cp6]/46,XY[19]. To the best of our knowledge, this is the first report on plasma cell myeloma with a variant Burkitt-type t(8;22)(q24;q11) in the Korean patient. A review of 11 such cases in the literature, including the present case, implicated that plasma cell myeloma with t(8;22)(q24;q11) might be related to advanced stage and poor prognosis.

SOLITARY PLASMA CELL MYELOMA ON ANTERIOR MAXILLA: A CASE REPORT

A case of POEMS syndrome presenting as primary adrenocortical insufficiency associated with plasma cell myeloma / 대한내과학회지

POEMS syndrome is multisystemic disorder characterized polyneuropathy, organomegaly, endocrinopathies, monoclonal gammapathy. A 38 year-old man visited our hospital because of progressive general weakness, hyperpigmentation. We diagnosed him as idiopathic primary adrenal insufficiency. The symptoms of general weakness were improved after steroid replacement therapy. But after 6 months, he revisited ER with chief complaints of abdominal distension and edema in lower extremities. He had also polyneuropathy, hepatosplenomegaly, IgA light chain monoclonal gammopathy, hyperpigmentation and thickening of the skin. Bone marrow biopsy showed that the plasma cells, mainly mature forms, are about 40% of all nucleated elements and on tissue section. We think this is the first case of POEMS syndrome presenting as primary adrenocortical insufficiency in Korea, so we report the case with reviews of other literatures. And we recommend performing a rapid ACTH stimulation test in all POEMS syndrome in order to detect adrenal insufficiency.