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1.
Rev. cuba. hematol. inmunol. hemoter ; 36(3): e1202, jul.-set. 2020. graf
Article in Spanish | LILACS, CUMED | ID: biblio-1156445

ABSTRACT

Introducción: La macroglobulinemia de Waldenström constituye una neoplasia hematológica del grupo de las gammapatías monoclonales, que incluye síntomas sistémicos y relacionados al incremento de la paraproteína M. Objetivo: Describir un caso de amiloidosis cardiaca asociada a macroglobulinemia. Caso clínico: Paciente masculino que fue admitido por astenia, disfonía, y durante su evolución desarrolló disnea progresiva, insuficiencia cardiaca y efusión pleural. Adicionalmente, la ecocardiografía mostró patrón granular miocárdico, y la biopsia pleural resultó positiva para la tinción rojo congo. Posteriormente, recibió tratamiento con bortezomib, dexametasona y rituximab con evolución favorable. Conclusiones: En esta enfermedad el diagnóstico temprano es una ventaja importante para la supervivencia. Es por esa razón, que su manejo es paliativo de las manifestaciones cardiacas. El presente caso pone en manifiesto un reto diagnóstico, en el cual se deben tomar en cuenta las etiologías menos frecuentes de insuficiencia cardiaca(AU)


Introduction: Waldenström's macroglobulinemia is a hematological neoplasm belonging to the group of monoclonal gammopathies, which includes systemic symptoms and those related to an increase in M paraprotein. Objective: To describe a case of cardiac amyloidosis associated with macroglobulinemia. Clinical case: Male patient who was admitted for asthenia, dysphonia, and who, during his evolution, developed progressive dyspnea, heart failure and pleural effusion. Additionally, echocardiography showed myocardial granular pattern, while pleural biopsy was positive for Congo red staining. Subsequently, he received treatment with bortezomib, dexamethasone and rituximab, with favorable evolution. Conclusions: In this disease, early diagnosis is an important advantage for survival. Therefore, its management is palliative of cardiac manifestations. The present case shows a diagnostic challenge, in which the less frequent etiologies of heart failure must be taken into account(AU)


Subject(s)
Humans , Male , Aged , Early Diagnosis , Survivorship , Amyloidosis/complications , Amyloidosis/drug therapy , Waldenstrom Macroglobulinemia/diagnosis , Congo Red/analysis , Amyloidosis/diagnostic imaging
2.
Article in English | WPRIM | ID: wpr-762462

ABSTRACT

BACKGROUND: JL1, a CD43 epitope and mucin family cell surface glycoprotein, is expressed on leukemic cells. An anti-JL1 antibody combined with a toxic substance can have targeted therapeutic effects against JL1-positive leukemia; however, JL1 expression on bone marrow (BM) lymphoma cells has not been assessed using flow cytometry. We investigated JL1 expression on BM lymphoma cells from patients with non-Hodgkin lymphoma (NHL) to assess the potential of JL1 as a therapeutic target. METHODS: Patients with BM involvement of mature B-cell (N=44) or T- and natural killer (NK)-cell (N=4) lymphomas were enrolled from May 2015 to September 2016. JL1 expression on BM lymphoma cells was investigated using flow cytometry. Clinical, pathological, and cytogenetic characteristics, and treatment responses were compared according to JL1 expression status. RESULTS: Of the patients with NHL and BM involvement, 37.5% (18/48) were JL1-positive. Among mature B-cell lymphomas, 100%, 38.9%, 33.3%, 100%, and 25.0% of Burkitt lymphomas, diffuse large B-cell leukemias, mantle cell leukemias, Waldenstrom macroglobulinemia, and other B-cell lymphomas, respectively, were JL1-positive. Three mature T- and NK-cell NHLs were JL1-positive. JL1 expression was associated with age (P=0.045), complete response (P=0.004), and BM involvement at follow-up (P=0.017), but not with sex, performance status, the B symptoms, packed marrow pattern, cytogenetic abnormalities, or survival. CONCLUSIONS: JL1 positivity was associated with superior complete response and less BM involvement in NHL following chemotherapy.


Subject(s)
B-Lymphocytes , Bone Marrow , Burkitt Lymphoma , Chromosome Aberrations , Cytogenetics , Drug Therapy , Flow Cytometry , Follow-Up Studies , Humans , Leukemia , Leukemia, B-Cell , Lymphoma , Lymphoma, B-Cell , Lymphoma, Non-Hodgkin , Membrane Glycoproteins , Mucins , Therapeutic Uses , Waldenstrom Macroglobulinemia
3.
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
4.
Rev. méd. Chile ; 147(3): 275-280, mar. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1004347

ABSTRACT

Background: Waldenström macroglobulinemia (WM) is an uncommon indolent B-cell lymphoma, due to the proliferation of lymphoplasmacytic cells, and secretion of a monoclonal IgM protein. Aim: To evaluate the clinical characteristics, management and results of treatment of patients with WM at a public hospital in Chile. Patients and Methods: Review of medical records of 31 patients aged 43 to 85 years (16 males) with WM diagnosed between 2002 and 2017. Clinical features and survival were recorded. Results: All patients had bone marrow compromise, and 31%, extranodal involvement. According to the International Prognostic Score System for WM (IPSSWM) 16, 58 and 26% were at low, intermediate and high risk, respectively. Twenty-five patients (81%) were treated, 32% with plasmapheresis and 36% with rituximab. Four cases (16%) achieved complete remission. Median follow up was 35 months (range 6-159). Estimated overall survival (OS) at 5 and 10 years was 74% and 53%, respectively. According to IPSSWM, the estimated five-year OS was 80, 92 and 39%, for low, intermediate and high-risk patients, respectively. Conclusions: OS was similar to that reported abroad, except for low risk patients, probably due to the low number of cases and short follow up. An improved survival should be expected with the routine use of immunochemotherapy.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Aged, 80 and over , Waldenstrom Macroglobulinemia/diagnosis , Vincristine , Biopsy , Bone Marrow/pathology , Prednisone/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Chile/epidemiology , Survival Rate , Retrospective Studies , Treatment Outcome , Waldenstrom Macroglobulinemia/mortality , Waldenstrom Macroglobulinemia/drug therapy , Cyclophosphamide/therapeutic use , Rituximab/therapeutic use , Antineoplastic Agents, Immunological/therapeutic use
6.
ARS med. (Santiago, En línea) ; 44(1): 46-50, 2019. ilus
Article in Spanish | LILACS | ID: biblio-1046760

ABSTRACT

Introducción: la Macroglobulinemia de Waldenström (MW) es un tipo raro de linfoma de células B caracterizado por la proliferación de células linfoplasmocíticas que secretan altas cantidades de inmunoglobulinas M (IgM) monoclonales. Puede presentarse con una amplia gama de síntomas, entre ellos los derivados del aumento de la viscosidad plasmática. Métodos: reportamos un caso de MW que presentó una trombosis de la vena central de la retina (TVCR) bilateral en el contexto de un síndrome de hiperviscosidad (SHV). El objetivo de este trabajo fue revisar algunos aspectos clínicos de la MW, con especial énfasis en el SHV y la TVCR. Resultados: las manifestaciones clínicas y de laboratorio de la MW son inespecíficas y comunes a otras neoplasias hematológicas. El SHV se produce cuando los niveles de IgM sobrepasan los 3 mg/dL, situación que ocurre en un 15-30 por ciento de los pacientes. La TVCR es una complicación grave y rara asociada al SHV, presentándose típicamente como una pérdida de agudeza visual indolora. El fondo de ojo exhibe signos característicos, como tortuosidad venosa y hemorragias retinales hasta la periferia, y la angiografía con fluorosceína y la OCT pueden orientar al diagnóstico y guiar el manejo. El tratamiento incluye la plasmaféresis y tratamientos oftalmológicos basados en agentes antioangiogénicos. Conclusión: la MW es una enfermedad incurable. Sin embargo, es importante sospechar una TVCR en pacientes que debutan con una pérdida de agudeza visual, ya que existen tratamientos efectivos en el manejo de esta complicación. (AU)


Introduction: Waldenstrom's Macroglobulinemia (WM) is a rare type of B-cell lymphoma characterized by proliferation of lymphoplasmocitarian cells that secrete high amounts of monoclonal immunoglobulin M (IgM). It may present with a wide range of symptoms, including the ones that derivatives of the increase in plasma viscosity. Methods: we report a case of WM that presented a bilateral central retinal vein occlusion (CRVO) in the context of a hyperviscosity syndrome (HVS). The objective of this study was to review clinical aspects of the WM, with special emphasis on the HVS and the CRVO. Results: the WM laboratory and clinical manifestations are common to other hematological malignancies. The HVS occurs when levels of IgM exceed 3 mg/dL, situation that occurs in 15-30 percent of patients. The CRVO is a serious and rare complication associated with the HVS, typically presented as a painless visual acuity loss. The fundoscopic examination exhibits characteristic signs, such as venous tortuousity and retinal hemorrhages in all four quadrants and most numerous in the periphery; angiography with fluoroscein and the OCT can orientate the diagnosis and guide the management. The treatment includes plasmapheresis and other ophthlamological treatments based on antiangiogenic agents. Conclusion: the WM is an incurable disease. However, it is important to suspect a CRVO in patients who made their debut with a loss of visual acuity, since there are effective treatments in the management of this complication.(AU)


Subject(s)
Humans , Retinal Vein Occlusion , Waldenstrom Macroglobulinemia , Therapeutics , Viscosity
7.
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
8.
Journal of Experimental Hematology ; (6): 1220-1224, 2018.
Article in Chinese | WPRIM | ID: wpr-689502

ABSTRACT

The 2016 world health organization (WHO) classification of B cell chronic lymphoproliferative disease (B-CLPD) includes chronic lymphocytic leukemia (CLL), B prolymphocytic leukemia, (B-PLL), hairy cell leukemia (HCL), marginal zone lymphoma (MZL), follicular lymphoma (FL), mantle cell lymphoma (MCL), Waldenstrom macroglobulinemia (LPL/WM). All the above-mentioned diseases are partially similar in cell morphology, immunophenotype and molecular genetics, but significantly different in treatment and prognosis. Currently, many new drugs targeted at cell cycle and apoptosis pathway, such as proteasome inhibitor immune modulators and histone deacetylase inhibitors, have achieved encouraging results in B-CLPD, which bring new hope for patients with B-CLPD. The review will discuss the progress in diagnosis and treatment of B-CLPD in recent years.


Subject(s)
B-Lymphocytes , Humans , Immunophenotyping , Leukemia, Lymphocytic, Chronic, B-Cell , Lymphoma, Mantle-Cell , Waldenstrom Macroglobulinemia
9.
Blood Research ; : 189-197, 2018.
Article in English | WPRIM | ID: wpr-716976

ABSTRACT

BACKGROUND: The incidence of lymphoplasmacytic lymphoma (LPL) is lower in Asian than in Western populations. Few studies have described the clinical features and treatment outcomes of patients with LPL, including non-IgM LPL, in East Asia. METHODS: We retrospectively analyzed patients diagnosed with LPL at Asan Medical Center between January 2001 and March 2016. We evaluated the clinical features and survival outcomes of patients with LPL and non-IgM LPL and compared these data with those of patients with LPL/Waldenström's macroglobulinemia (WM). RESULTS: The median age at diagnosis of patients with LPL was 61.5 years (range, 34–77 yr); most patients were male (91%). Approximately three-quarters of the 22 patients with LPL were in the low or intermediate risk groups according to the International Prognostic Scoring System for Waldenström's Macroglobulinemia classification. The median follow-up duration was 75 months [95% confidence interval (CI), 48–102 mo], and the median overall survival (OS) was 81 months (95% CI, 0–167 mo). The number of patients in the non-IgM LPL group who exhibited extramedullary involvement was higher than in the LPL/WM group. OS of the LPL/WM group was improved compared with that of the non-IgM LPL group [median not reached vs. 10.0 mo (95% CI, 0–36.7); P=0.05]. CONCLUSION: We present a single-center experience of 22 patients with LPL, including a non-IgM cohort, in Korea. The treatment of non-IgM LPL was heterogeneous, and patients with non-IgM LPL showed a higher 5-year mortality rate and more adverse prognostic factors than those with LPL/WM.


Subject(s)
Asian Continental Ancestry Group , Classification , Cohort Studies , Diagnosis , Far East , Follow-Up Studies , Humans , Incidence , Korea , Lymphoma , Male , Mortality , Retrospective Studies , Waldenstrom Macroglobulinemia
10.
Blood Research ; : 117-122, 2018.
Article in English | WPRIM | ID: wpr-714933

ABSTRACT

BACKGROUND: Waldenström Macroglobulinemia (WM) is a rare subtype of indolent B-cell lymphoma, and prospective randomized studies on WM are scarce. The R-CHOP therapy [rituximab (R), cyclophosphamide, hydroxy-doxorubicin, vincristine, and prednisone] is a popular and recommended regimen for primary therapy, prescribed by several treatment guidelines for WM. However, treatment with R-CHOP is accompanied by severe myelosuppression and high rates of peripheral neuropathy. Therefore, we retrospectively evaluated the efficacy and toxicity of half-dose CHOP combined with R as a primary therapy for WM. METHODS: Patients with untreated symptomatic WM, treated at the Disaster Medical Center between April 2011 and September 2016, were retrospectively analyzed after administration of 6 cycles of half-dose R-CHOP for every 3 weeks. The response, median time to response, best response, progression-free survival, overall survival, and toxicities were evaluated. RESULTS: Of the 20 WM patients analyzed, 16 (80%) received half-dose R-CHOP without vincristine, and 13 (65%) responded to the treatment. With a median follow-up duration of 26.3 months, the 2-year progression-free survival and 2-year overall survival rates were 70 and 93.3%, respectively. The median time to response and best response were 6 and 9.9 weeks, respectively. Grade 3/4 leukocytopenia, neutropenia, febrile neutropenia, and Grade 1 peripheral neuropathy developed in 32, 37, 0, and 21% of patients, respectively. CONCLUSION: The half-dose R-CHOP is an effective and well-tolerated primary therapy for WM. To the best of our knowledge, this is the first study reporting the use of a reduced-dose R-CHOP regimen for the primary treatment of WM.


Subject(s)
Cyclophosphamide , Disasters , Disease-Free Survival , Febrile Neutropenia , Follow-Up Studies , Humans , Leukopenia , Lymphoma, B-Cell , Neutropenia , Peripheral Nervous System Diseases , Prospective Studies , Retrospective Studies , Rituximab , Survival Rate , Vincristine , Waldenstrom Macroglobulinemia
12.
Article in English | WPRIM | ID: wpr-633748

ABSTRACT

INTRODUCTION: Indolent lymphoma (IL) is a slowly growing lymphoma, generally refractory to conventional chemotherapy. There are several types of IL, which includes follicular lymphoma (FL), marginal zone lymphoma (MZL), small lymphocytic lymphoma (SLL), mantle cell lymphoma (MCL), and waldenstrom macroglobulinemia/ lymphoplasmacytic lymphoma (WM/LPL). Presently, there are no known data in the Philippines on IL. This study is done to determine the clinico-pathologic profile and outcomes of Filipino patients with IL. METHODS: This study is a retrospective chart review of outpatient department cases of IL seen at the Philippine General Hospital-Cancer Institute from January 2009 to January 2016. The following were documented: age; gender; primary location; presence or absence of B symptoms; type of IL; Ann-arbor stage; prognostic indices for FL and MCL; and staging with bone marrow aspiration and whole body CT scan. Treatment intervention and clinical outcomes were documented. RESULTS: This study showed that SLL was the most common IL. Most were elderly (>40 years old); male; lacked B symptoms; limited disease; and primary location at or near the orbital area. MCL were seen in all risk groups. Follicular lymphoma (FL) were mostly low risk and had grade one histology. Majority had disease control regardless of treatment intervention. Most patients with recurrence/progression after initial treatment had limited disease but were understaged. Most of the patients were not staged with bone marrow aspiration or whole body computed tomography. CONCLUSION: The results of this study are mostly consistent with known literature on IL. Absence of B symptoms and limited disease may indicate a low-grade histology. Observation was the most common option for asymptomatic patients.  


Subject(s)
Humans , Male , Female , Aged , Middle Aged , Adult , Young Adult , Lymphoma, Follicular , Leukemia, Lymphocytic, Chronic, B-Cell , Waldenstrom Macroglobulinemia , Bone Marrow , Prefrontal Cortex , Tomography
13.
Article in Korean | WPRIM | ID: wpr-203539

ABSTRACT

Neutrophilic dermatoses comprise a wide spectrum of inflammatory diseases with overlapping features characterized histologically by the presence of an aseptic neutrophilic infiltrate in the epidermis, dermis, and/or hypodermis and are often associated with systemic inflammatory and neoplastic disorders. Here we report a case of a 75-year-old man who developed painful indurated plaques with a vesicular, bullous, and even hemorrhagic appearance similar to cellulitis on his right leg along with a fever that raised his body temperature up to 39℃ and malaise.


Subject(s)
Aged , Body Temperature , Cellulitis , Dermis , Epidermis , Fever , Humans , Leg , Neutrophils , Skin Diseases , Subcutaneous Tissue , Sweet Syndrome , Waldenstrom Macroglobulinemia
14.
Article in English | WPRIM | ID: wpr-43199

ABSTRACT

We report a case of Waldenström's macroglobulinemia (WM) treated using clarithromycin (CAM) and prednisolone (PSL). An 84-year-old woman was admitted to our hospital for bleeding after a tooth extraction and hematuria. Computed tomography showed multiple ill-defined nodules in the omentum (omental cake). Although the cause of the omental cake remained unclear, the patient was diagnosed with WM, based on the detection of M-protein of immunoglobulin (Ig) M in serum and lymphoplasmacytes in bone marrow. The bleeding tendency in the patient may have been due to acquired hemophilia and/or hyper IgM-induced platelet dysfunction. The patient was treated using CAM (800 mg/day) and PSL (10 mg/day). As a result, IgM levels gradually decreased. Because the omental cake contracted along with improvement in IgM, it was thought to be lymphoplasmacytic lymphoma-like lymphoma. This case shows that treatment using CAM and PSL may be effective in some cases of WM.


Subject(s)
Aged, 80 and over , Blood Platelets , Bone Marrow , Clarithromycin , Female , Hematuria , Hemophilia A , Hemorrhage , Humans , Immunoglobulin M , Immunoglobulins , Lymphoma , Omentum , Prednisolone , Tooth Extraction , Waldenstrom Macroglobulinemia
15.
An. bras. dermatol ; 91(2): 236-238, Mar.-Apr. 2016. graf
Article in English | LILACS | ID: lil-781363

ABSTRACT

Abstract Waldenstrom’s macroglobulinemia is considered a lymphoma by the World Health Organization. Cutaneous lesions, particularly of a specific type, are rare occurring in 5% of patients. What draws attention in this case is the unusual cutaneous clinical manifestation and its location on the genitals, which has not been described in researched literature, therefore imposing differential diagnosis with other etiologies of genital ulcers.


Subject(s)
Humans , Male , Middle Aged , Penile Neoplasms/pathology , Skin Ulcer/pathology , Waldenstrom Macroglobulinemia , Skin/pathology , Fatal Outcome
16.
Blood Research ; : 181-186, 2016.
Article in English | WPRIM | ID: wpr-209255

ABSTRACT

BACKGROUND: Recent studies have identified a high prevalence of the MYD88 L265P mutation in lymphoplasmacytic lymphoma (LPL)/Waldenstrom macroglobulinemia (WM) cases, whereas low frequencies have been observed in other B cell non-Hodgkin lymphomas (NHLs). METHODS: We evaluated the sensitivity of the mutant enrichment 3'-modified oligonucleotide (MEMO)-PCR technique, a new detection method. We examined the MYD88 L265P mutation in a series of Korean patients with LPL/WM and other B cell NHLs in bone marrow aspirates, using the MEMO-PCR technique. RESULTS: The sensitivity of MEMO-PCR was estimated to be approximately 10-16.7%. MYD88 L265P was detected in 21 of 28 LPL cases (75%) and only three of 69 B cell NHL cases (4.3%). CONCLUSION: Although MEMO-PCR had relatively low sensitivity, we confirmed the high prevalence of the MYD88 L265P mutation in Korean LPL patients. Our study suggests the diagnostic value of MYD88 L265P for differentiating B-cell NHLs.


Subject(s)
B-Lymphocytes , Bone Marrow , Humans , Lymphoma , Lymphoma, Non-Hodgkin , Methods , Prevalence , Waldenstrom Macroglobulinemia
17.
Article in Korean | WPRIM | ID: wpr-90323

ABSTRACT

PURPOSE: The authors report a case of bilateral simultaneous central retinal vein occlusion caused by Waldenstrom's macroglobulinemia. CASE SUMMARY: A 65-year-old man presented to our department complaining of decreased visual acuity for the duration of about 6 months. On his initial visit, best-corrected visual acuity was 0.02 in the right eye and 0.06 in the left eye. Based on the findings of a funduscopic examination, the patient had bilateral diffuse retinal hemorrhages, dilated tortuous veins, and macular edema. He had experienced recurrent spontaneous epistaxis 6 months previously and had undergone treatments such as intravitreal bevacizumab injection and intravitreal dexamethasone implantation at another hospital. Laboratory tests at that hospital showed anemia and hyperproteinemia, for which he was referred to our hemato-oncology department. Bone marrow biopsy was consistent with Waldenstrom's macroglobulinemia/lymphoplasmacytoid lymphoma, and he was treated with systemic chemotherapy. One year after the systemic chemotherapy, his best-corrected visual acuity was 0.15 in the right eye and 0.6 in the left eye. Funduscopy showed decreased bilateral retinal hemorrhages and macular edema. CONCLUSIONS: When simultaneous bilateral central retinal vein occlusion occurs in a patient with no other underlying disease such as hypertension or diabetes, it might be a sign of serum hyperviscosity, and there should be a very high level of suspicion for presence or progression of systemic disease. If such a disease is properly and timely diagnosed, effective early systemic evaluation and therapy can be administered, and it is important to have initial general treatment as well as ophthalmic treatment.


Subject(s)
Aged , Anemia , Bevacizumab , Biopsy , Bone Marrow , Dexamethasone , Drug Therapy , Epistaxis , Humans , Hypertension , Lymphoma , Macular Edema , Retinal Hemorrhage , Retinal Vein , Veins , Visual Acuity , Waldenstrom Macroglobulinemia
20.
Chinese Journal of Hematology ; (12): 493-496, 2015.
Article in Chinese | WPRIM | ID: wpr-282000

ABSTRACT

<p><b>OBJECTIVE</b>To observe the clinical and biological characteristics of Non-IgM-secreting lymphoplasmacytic lymphoma (LPL) and draw the differences between non-IgM LPL and Waldenström macroglobulinemia (WM).</p><p><b>METHODS</b>Records of 13 patients with non-IgM LPL were retrospectively analyzed between January 2000 and December 2013. The cytogenetic aberrations were detected by fluorescence in situ hybridisation (FISH).</p><p><b>RESULTS</b>In the cohort, 7 males and 6 females with a median age of 63 years (range 43 to 74), two patients were IgA secreting, 6 with IgG secreting and 5 patients without monoclonal globulin. The major complaint at diagnosis included anemia associated symptom (53.8%), mucocutaneous hemorrhage and superficial lymphadenopathy (15.4%). Eight patients had B symptom at diagnosis. All of the 13 patients had bone marrow involvement and anemia, and 10 patients had 2 or 3 lineage cytopenia. In 5 patients with available immunophenotypic data, all expressed CD19, CD20, CD22 and CD25, but missed the expression of CD10, CD103 and CD38. Two cases had CD5 or sIgM positive alone. Another 2 patients were CD23 or CD11c positive and 3 patients were FMC7 positive. Cytogenetic aberrations had been detected by FISH in 7 patients, but only two (28.6%) patients had aberrations with del(6q).</p><p><b>CONCLUSION</b>The clinical and biological characteristics had no significantly difference between non-IgM LPL and WM.</p>


Subject(s)
Adult , Aged , Antigens, CD , Chromosome Aberrations , Female , Humans , Immunoglobulin M , In Situ Hybridization, Fluorescence , Integrin alpha Chains , Leukemia, Lymphocytic, Chronic, B-Cell , Male , Middle Aged , Retrospective Studies , Waldenstrom Macroglobulinemia
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