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1.
Rinsho Ketsueki ; 60(10): 1449-1454, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31695006

RESUMO

The clinical course of chronic lymphocytic leukemia (CLL) is often complicated by autoimmune cytopenia (AIC). Here we report a case of a 69-year-old woman with CLL complicated by monoclonal immunoglobulin deposition disease (MIDD) and AIC. The patient was diagnosed with CLL at the age of 63 years and treated with chemotherapy including rituximab and/or fludarabine owing to the development of anemia, multiple lymphadenopathy, and B symptoms at the age of 66 years. Furthermore, pancytopenia and renal failure developed at the age of 68 years. Consequently, the patient was admitted owing to dyspnea. Because of no apparent signs of CLL progression, we concluded that pancytopenia was due to AIC (autoimmune granulocytopenia and thrombocytopenia) and renal anemia. MIDD was diagnosed based on renal histology and detection of IgM and λ chain immunoglobulin deposits on glomeruli and tubules, which were presumed to be derived from CLL cells. The patient was treated with ibrutinib in order to reduce monoclonal protein levels. AIC improved concurrently with IgM reduction 1 month later. Supportive care, involving transfusion and granulocyte colony-stimulating factor, was not required approximately 3 months after initiating ibrutinib treatment. In contrast, MIDD did not improve and maintenance hemodialysis was required. Owing to its antitumor and immunomodulatory effects, ibrutinib may contribute to improve CLL-associated AIC.


Assuntos
Anticorpos Monoclonais , Doenças Autoimunes/tratamento farmacológico , Leucemia Linfocítica Crônica de Células B/complicações , Pirazóis/uso terapêutico , Pirimidinas/uso terapêutico , Idoso , Feminino , Humanos , Rituximab
2.
Ann Clin Lab Sci ; 49(5): 671-674, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31611213

RESUMO

More than 100 cases harboring both myeloproliferative neoplasms (MPN) and chronic lymphocytic leukaemia (CLL) have been reported, suggesting that the two diseases can coexist in one patient. However, the mechanism by which this phenomenon is caused remains unclear. In this study, one patient with polycythemia vera (PV) and CLL is examined. Identifications of the JAK2V617F and P53H179L/ P53R209fs mutations were obtained via targeted next generation sequencing. Furthermore, B lymphocytes and neutrophils were separated from peripheral blood. This led to the discovery that JAK2V617F occurs in neutrophil's compartment, with P53 mutations emerging in B lymphocytes. These results indicate that PV and CLL are independent clonal diseases in this case, rather than phenotypes derived from one clone.


Assuntos
Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/patologia , Policitemia Vera/complicações , Policitemia Vera/patologia , Linfócitos B/patologia , Células Clonais , Humanos , Masculino , Pessoa de Meia-Idade , Mutação/genética , Neutrófilos/patologia
4.
Orv Hetil ; 160(38): 1487-1494, 2019 Sep.
Artigo em Húngaro | MEDLINE | ID: mdl-31537095

RESUMO

Immune status was investigated in 186 patients with chronic lymphoid leukaemia between January 2012 and March 2015. Incidences of infections and mortality were analysed in patients who did not receive prophylactic immunoglobulin therapy. Immunoglobulin G (IgG) levels were normal (7-17.8 g/L) or decreased in 62.37% and 35.48% of patients, respectively. We measured high immunoglobulin levels only in a few cases (2.15%). Immunoglobulin levels became increasingly lower in more advanced disease stages (Rai stages). The number of infections was inversely proportional to that. Hypogammaglobulinaemia proved to be more important than disease progression in terms of the development of infections. The most common infections were upper respiratory tract (33.07%) and sepsis (18.90%). Two months after chemotherapy, initially normal immunoglobulin levels decreased by an average of 21%, and at the same time the incidence of infections increased. The most common cause of death was sepsis: 30% occurred at low immunoglobulin levels, while 20% at normal immunoglobulin levels. According to literature, prophylactic immunoglobulin treatment is indicated in patients with chronic lymphoid leukaemia and immunodeficiency for decreasing both morbidity and mortality. According to recommendations in literature, replacement treatment must be administered in severe or moderately severe recurrent bacterial infections. Immunoglobulin prophylaxis may be provided as low dose (10 g), fix dose (18 g) or individually customized higher dose (300-400 mg/kg body weight) treatment. According to recommendations, higher dose immunoglobulin prophylaxis, administered every three weeks on six occasions, is more efficient when customized. With this dose, infection-free condition may be achieved in 50% of patients. Orv Hetil. 2019; 160(38): 1487-1494.


Assuntos
Agamaglobulinemia/tratamento farmacológico , Agamaglobulinemia/mortalidade , Imunoglobulinas Intravenosas/uso terapêutico , Imunoglobulinas/administração & dosagem , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Sepse/mortalidade , Agamaglobulinemia/complicações , Relação Dose-Resposta a Droga , Feminino , Humanos , Hungria/epidemiologia , Imunoglobulina G/sangue , Imunoglobulinas Intravenosas/administração & dosagem , Controle de Infecções , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/mortalidade , Masculino , Sepse/diagnóstico , Resultado do Tratamento
6.
BMJ Case Rep ; 12(7)2019 Jul 08.
Artigo em Inglês | MEDLINE | ID: mdl-31289156

RESUMO

A 69-year-old Caucasian woman presented with chronic lymphocytic leukaemia (CLL; stage 1-Rai System), significant oropharyngeal lymphoid enlargement, snoring and fatigue. Overnight polysomnography revealed moderately severe obstructive sleep apnoea (OSA), which was managed successfully with oral appliance therapy with resolution of snoring and daytime fatigue. Structural abnormalities of the upper airways are known to cause OSA. Airway narrowing can result from bony structural abnormalities, nasopharyngeal growth, soft tissue redundancy, macroglossia, malignant and benign growth of the upper aero-digestive tract, and adenotonsilar enlargement. Clinicians should be encouraged to consider a diagnosis of OSA in patients with CLL when they present with symptoms of worsening fatigue.


Assuntos
Tratamento Conservador/métodos , Leucemia Linfocítica Crônica de Células B/complicações , Desenho de Aparelho Ortodôntico/instrumentação , Apneia Obstrutiva do Sono/etiologia , Idoso , Feminino , Humanos , Orofaringe/patologia , Polissonografia , Apneia Obstrutiva do Sono/fisiopatologia , Ronco/etiologia , Resultado do Tratamento
7.
BMJ Case Rep ; 12(7)2019 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-31308179

RESUMO

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an extremely rare haematological malignancy defined by concurrent expression of CD4, CD56, BCL-2 and CD123. The disease has a very poor prognosis and there are no well-established treatment guidelines. We describe a case of BPDCN in a 65-year-old female patient with myeloproliferative disorder (essential thrombocythemia) and chronic lymphocytic leukaemia. She presented with rapidly progressive facial and scalp lesions. Skin biopsy confirmed BPDCN and the imaging revealed widespread disease. Patient was started on hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) and intrathecal methotrexate. Due to progression on initial treatment, she was treated with decitabine and venetoclax (BCL-2 inhibitor). However, patient continued to deteriorate and died after 4 months from initial diagnosis. We emphasise on the clinical features, emerging treatment modalities and prognosis of BPDCN.


Assuntos
Células Dendríticas , Leucemia Linfocítica Crônica de Células B/complicações , Neoplasias Cutâneas/tratamento farmacológico , Trombocitemia Essencial/complicações , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia , Linfócitos T CD4-Positivos/metabolismo , Antígeno CD56/metabolismo , Diagnóstico Diferencial , Neoplasias Faciais/tratamento farmacológico , Neoplasias Faciais/patologia , Evolução Fatal , Feminino , Neoplasias de Cabeça e Pescoço/tratamento farmacológico , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Doenças Raras , Couro Cabeludo , Neoplasias Cutâneas/patologia
9.
Eur Radiol ; 29(12): 6911-6921, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31236702

RESUMO

OBJECTIVE: To test the hypothesis that both indolent and aggressive chronic lymphocytic leukemia (CLL) can be differentiated from diffuse large B cell lymphoma (DLBCL) of Richter syndrome (RS) by CT texture analysis (CTTA) of involved lymph nodes. MATERIAL AND METHODS: We retrospectively included 52 patients with indolent CLL (26/52), aggressive CLL (8/52), and DLBCL of RS (18/52), who underwent standardized contrast-enhanced CT. In main lymphoma tissue, VOIs were generated from which CTTA features including first-, second-, and higher-order textural features were extracted. CTTA features were compared between the entire CLL group, the indolent CLL subtype, the aggressive CLL subtype, and DLBCL using a Kruskal-Wallis test. All p values were adjusted after the Bonferroni correction. ROC analyses for significant CTTA features were performed to determine cut-off values for differentiation between the groups. RESULTS: Compared with DLBCL of RS, CTTA of the entire CLL group showed significant differences of entropy heterogeneity (p < 0.001), mean intensity (p < 0.001), mean average (p = 0.02), and number non-uniformity gray-level dependence matrix (NGLDM) (p = 0.03). Indolent CLL significantly differed for entropy (p < 0.001), uniformity of heterogeneity (p = 0.02), mean intensity (p < 0.001), and mean average (p = 0.01). Aggressive CLL showed significant differences in mean intensity (p = 0.04). For differentiation between CLL and DLBCL of RS, cut-off values for mean intensity and entropy of heterogeneity were defined (e.g., 6.63 for entropy heterogeneity [aggressive CLL vs. DLBCL]; sensitivity 0.78; specificity 0.63). CONCLUSIONS: CTTA features of ultrastructure and vascularization significantly differ in CLL compared with that in DLBCL of Richter syndrome, allowing complementary to visual features for noninvasive differentiation by contrast-enhanced CT. KEY POINTS: • Richter transformation of CLL into DLBCL results in structural changes in lymph node architecture and vascularization that can be detected by CTTA. • First-order CT textural features including intensity and heterogeneity significantly differ between both indolent CLL and aggressive CLL and DLBCL of Richter syndrome. • CT texture analysis allows for noninvasive detection of Richter syndrome which is of prognostic value.


Assuntos
Sarcoma de Células Dendríticas Foliculares/patologia , Leucemia Linfocítica Crônica de Células B/patologia , Linfoma Difuso de Grandes Células B/patologia , Idoso , Diferenciação Celular , Sarcoma de Células Dendríticas Foliculares/complicações , Sarcoma de Células Dendríticas Foliculares/diagnóstico por imagem , Feminino , Humanos , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/diagnóstico por imagem , Linfonodos/patologia , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Sensibilidade e Especificidade , Síndrome , Tomografia Computadorizada por Raios X/métodos
10.
Blood Coagul Fibrinolysis ; 30(5): 239-242, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31157683

RESUMO

: Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic condition that poses both a diagnostic and a therapeutic challenge. Here we report a singular case of AVWS with two associated conditions, small lymphocytic lymphoma (SLL) and Sjögren's syndrome. The patient presented with recurrent and severe digestive bleeding that forced us to raise a curative attempt of AVWS. A first immunosuppressive therapy with immunoglobulins was unsuccessful and it was later decided to treat lymphoproliferative entity with bendamustine and rituximab effectively achieving SLL and AVWS remission. On the basis of our case and through literature review, we discuss potential strategies to achieve AVWS remission when it appears in the setting of several causative associated conditions.


Assuntos
Leucemia Linfocítica Crônica de Células B/complicações , Síndrome de Sjogren/complicações , Doenças de von Willebrand/complicações , Idoso , Antineoplásicos Alquilantes/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Cloridrato de Bendamustina/uso terapêutico , Feminino , Hemorragia/etiologia , Hemorragia/terapia , Humanos , Imunoglobulinas/uso terapêutico , Leucemia Linfocítica Crônica de Células B/terapia , Rituximab/uso terapêutico , Síndrome de Sjogren/terapia , Doenças de von Willebrand/terapia
12.
Dermatol Online J ; 25(3)2019 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-30982302

RESUMO

Granuloma annulare (GA) is a fairly common inflammatory skin condition with a range of clinical subtypes. We describe an unusual case of unilateral GA confined to the thigh on a previously amputated limb. A man in his 80s with a past medical history of below-knee amputation of the left leg owing to severe leg ulcers from pyoderma gangrenosum, chronic lymphocytic leukemia, and dyslipidemia developed a slowly spreading eruption on the distal stump spreading proximally. On physical examination, he had numerous non-scaly violaceous papules and annular plaques from the stump to the lateral, medial, and anterior thigh. Histology confirmed a diagnosis of GA. The extensive, chronic lesions make this presentation of GA very unusual in that it shares features of both localized and generalized forms. Moreover, the temporal and spatial association with pyoderma gangrenosum is unique and may reflect a related inflammatory pathway.


Assuntos
Cotos de Amputação/patologia , Granuloma Anular/diagnóstico , Dermatoses da Perna/diagnóstico , Leucemia Linfocítica Crônica de Células B/complicações , Pioderma Gangrenoso/diagnóstico , Idoso de 80 Anos ou mais , Granuloma Anular/complicações , Granuloma Anular/patologia , Humanos , Dermatoses da Perna/complicações , Dermatoses da Perna/patologia , Masculino , Pioderma Gangrenoso/complicações , Pioderma Gangrenoso/cirurgia
13.
Blood Cells Mol Dis ; 77: 109-112, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31029024

RESUMO

Acquired von Willebrand syndrome (AVWS) is a rare, potentially fatal bleeding disorder caused by low activity of von Willebrand factor (VWF) in patients without congenital deficiency. The majority of adult cases are associated with hematological malignancy, including lymphoproliferative (48%) or myeloproliferative (15%) disorders (Federici et al., 2000). Both qualitative and quantitative defects occur, due to antibody-mediated clearance or functional interference, increased proteolysis, absorption to malignant cells or platelets, or increased shear stress due to valvular defects or mechanical vascular devices (Tiede et al., 2011). The predominant mechanism for decreased or absent VWF in malignancy is autoantibodies that are inhibitory to VWF function or shorten VWF survival (Kumar et al., 2002 [3]). Antibody-mediated clearance occurs through inactivating antibody directed towards VWF, antibody binding the non-active sites of VWF, and nonspecific antibodies that form circulating immune complexes with VWF, enhancing clearance by the reticuloendothelial system (Mannucci et al., 1984). Bleeding may be very difficult to treat due to reduced half-life of VWF-concentrates.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Leucemia Linfocítica Crônica de Células B/complicações , Doenças de von Willebrand/etiologia , Doenças de von Willebrand/terapia , Adulto , Terapia Combinada , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Masculino , Troca Plasmática , Quimeras de Transplante , Transplante Homólogo , Resultado do Tratamento , Doenças de von Willebrand/sangue , Doenças de von Willebrand/diagnóstico , Fator de von Willebrand/metabolismo
15.
BMC Res Notes ; 12(1): 202, 2019 Apr 02.
Artigo em Inglês | MEDLINE | ID: mdl-30940190

RESUMO

OBJECTIVE: To assess the clinical presentation and hematological profile among young (≤ 55 years) and old (> 55 years) chronic lymphocytic leukemia patients in Sudan. RESULT: In the present cross-sectional descriptive study, out of 110 cases studied, among them 31 (28.2%) were young (≤ 55 years) patients with mean age 48 years, and 79 (71.8%) were elder patients (> 55 years) with mean age 66 years, the overall mean age was 62.97 ± 12.06 with range (22-85 years), and 79 (71.8%) were males and 31 (28.2%) were females (M:F = 2.6:1) (P = 0.000). (7.3%) were asymptomatic, 61 (55.5%) presented with nonspecific complains. Generalized lymphadenopathy was seen in 52 (47.27%) with elder predominance (P = 0.03). Splenomegaly, hepatomegaly, thrombocytopenia and anemia were seen in 54 (49.1%), 14 (12.7%), 43 (39.1%) and 38 (34.5%) of patients respectively with male predominance. 54 (49.1%) and 42 (38.18%) of patients presented at Rai high risk and Binet C stages respectively with nearly same age and sex distribution. CLL in Sudan is a disease of elders, same as seen in literature, with high male to female ratio. In general hematological parameters means were noted to be distributed equally according to age and sex groups. Majority of patients were presented with nonspecific symptoms and nearly half of patients presented at late stages as reported in most developing countries.


Assuntos
Anemia , Hepatomegalia , Leucemia Linfocítica Crônica de Células B , Linfadenopatia , Esplenomegalia , Trombocitopenia , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Anemia/sangue , Anemia/epidemiologia , Anemia/etiologia , Feminino , Hepatomegalia/sangue , Hepatomegalia/epidemiologia , Hepatomegalia/etiologia , Humanos , Leucemia Linfocítica Crônica de Células B/sangue , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/epidemiologia , Linfadenopatia/sangue , Linfadenopatia/epidemiologia , Linfadenopatia/etiologia , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Esplenomegalia/sangue , Esplenomegalia/epidemiologia , Esplenomegalia/etiologia , Sudão/epidemiologia , Trombocitopenia/sangue , Trombocitopenia/epidemiologia , Trombocitopenia/etiologia , Adulto Jovem
16.
J Am Acad Dermatol ; 80(6): 1544-1549, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30981429

RESUMO

BACKGROUND: Paraneoplastic pemphigus (PNP) occurs more often in patients with hematologic malignancies (HMs) than in patients with solid cancer. Lung bronchiolitis obliterans (BO) is a severe complication of PNP. OBJECTIVE: To determine the precise clinical and biologic features of HM-associated PNP and identify factors associated with mortality and survival. METHODS: Systematic review of previously described cases of PNP associated with HMs. RESULTS: A total of 144 patients were included. The HMs were non-Hodgkin lymphoma (52.78%), chronic lymphocytic leukemia (22.92%), Castleman disease (18.60%), and other underlying hematologic malignancy (5.70%). The mortality rate was 57%, and most deaths occurred within the first year after the diagnosis of PNP. Multivariate analysis showed that (1) the presence of antienvoplakin antibodies and BO were significantly associated with death, and (2) a toxic epidermal necrolysis-like clinical pattern, bullous pemphigoid-like clinical pattern, and BO were significantly associated with decreased survival. LIMITATION: Only case reports with sufficient mortality data were included. CONCLUSION: PNP associated with HM has a high mortality rate. The toxic epidermal necrolysis-like and BO-associated forms are independent survival factors in PNP associated with HMs.


Assuntos
Neoplasias Hematológicas/complicações , Síndromes Paraneoplásicas/mortalidade , Pênfigo/mortalidade , Idoso , Biomarcadores , Biópsia , Bronquiolite Obliterante/etiologia , Hiperplasia do Linfonodo Gigante/complicações , Feminino , Humanos , Estimativa de Kaplan-Meier , Leucemia Linfocítica Crônica de Células B/complicações , Linfoma não Hodgkin/complicações , Masculino , Pessoa de Meia-Idade , Especificidade de Órgãos , Síndromes Paraneoplásicas/etiologia , Síndromes Paraneoplásicas/patologia , Pênfigo/etiologia , Pênfigo/patologia , Prognóstico , Modelos de Riscos Proporcionais , Fatores de Risco , Pele/química , Pele/patologia
17.
Vox Sang ; 114(5): 495-504, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30972770

RESUMO

BACKGROUND AND OBJECTIVES: Revised Icelandic guidelines proposed a restrictive haemoglobin (Hb) threshold of 70 g/l for red blood cell (RBC) transfusions in general, but 100 g/l for malignancies/bone marrow suppression. Chronic lymphocytic leukaemia (CLL) is frequently complicated by anaemia. The objective was to investigate RBC transfusion practices in CLL. MATERIALS AND METHODS: This retrospective nation-wide study utilized an Icelandic registry of CLL patients diagnosed between 2003 and 2016. Medical records were reviewed and haemoglobin transfusion triggers compared for two periods: Earlier (2003-2012) and latter (2013-2017). RESULTS: Two hundred and thirteen patients were diagnosed with CLL over the period whereof 77 (36·2%) received RBC transfusion(s). Median time from diagnosis to first transfusion was 2·2 years. Higher age, Rai stage 3/4 at diagnosis (P < 0·05) and chemotherapy (P < 0·001) were associated with increased odds of transfusions. Shorter time to first transfusion correlated with higher age (P < 0·001) and Rai stage (P = 0·02) at diagnosis. The mean Hb trigger was 90·4 and 81·2 in the earlier and latter period respectively (P = 0·01). This difference in Hb triggers was most pronounced in patients without documented bone marrow involvement, or 80·5 g/l compared to 93·5 g/l (P = 0·004). The median time from diagnosis to transfusion was longer in the latter period (2·9 years vs. 1·6 years, P = 0·01). After RBC transfusions the survival decreased significantly (P < 0·001). CONCLUSION: One-third of CLL patients received RBC transfusions but few were heavily transfused. Older age, Rai stage, and chemotherapy predicted RBC use. The Hb transfusion trigger decreased over time while time to first RBC transfusion increased. RBC transfusions predict poor survival.


Assuntos
Anemia/terapia , Transfusão de Eritrócitos/normas , Leucemia Linfocítica Crônica de Células B/complicações , Sistema de Registros , Adulto , Idoso , Idoso de 80 Anos ou mais , Anemia/complicações , Feminino , Hemoglobinas , Humanos , Islândia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
18.
Cancer Epidemiol ; 60: 128-133, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30986631

RESUMO

BACKGROUND: Transformation to aggressive lymphoma (Richter syndrome, RS) occurs in a substantial subset of patients who must discontinue targeted therapy for chronic lymphocytic leukemia (CLL). RS has an extremely poor prognosis. METHODS: Using the nation-wide database of The Cancer Registry of Norway of 7664 CLL patients registered between 1953-2012, we identified 107 patients experiencing RS. RESULTS: Seventy seven (72%) of RS patients were identified among 2631 CLL patients diagnosed between 2003-2012; diffuse large B-cell lymphoma (DLBCL) was identified in 65 (84%), Hodgkin lymphoma (HL) in 12 (16%) patients and the diagnosis was confirmed in 50 (65%) available biopsy specimens. The incidence rate in this period was 4.7/1000 person-years (95% CI: 3.8-5.9). The median survival from CLL diagnosis was 1.7 years (95% CI: 0.34-2.3) for RS patients while it was 10.3 years (95% CI: 9.5-10.9) for the remaining CLL patients. Male gender predominated among RS patients (69%) compared to CLL population (58%) and RS patients were diagnosed with CLL at a significantly younger age than the remaining patients (65 vs. 72 years). Median time from diagnosis of CLL to RS was 2 years (Range, 0-13 years). No CLL treatment was administered in 25 (33%) patients prior RS diagnosis; a median of 1 treatment line was administered to pretreated patients. The median duration of survival after RS diagnosis was 27 months (95% CI; 9-88). CONCLUSIONS: Collectively, RS was a rare complication of CLL in the chemoimmunotherapy era, occurred early in the CLL course in younger, and both treatment naïve and pretreated patients, and shortened survival substantially.


Assuntos
Leucemia Linfocítica Crônica de Células B/complicações , Linfoma Difuso de Grandes Células B/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Humanos , Leucemia Linfocítica Crônica de Células B/patologia , Masculino , Pessoa de Meia-Idade , Noruega , Prognóstico
20.
BMJ Case Rep ; 12(3)2019 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-30872333

RESUMO

A 46-year-old man presented with splenomegaly, abdominal adenopathy and profoundly elevated prothrombin time and partial thromboplastin time. He was diagnosed with marginal zone lymphoma (MZL) and small lymphocytic lymphoma, and the abnormal coagulation studies were secondary to the presence of a lupus anticoagulant. Optimal upfront therapy for MZL has not been established, and the incidence of antiphospholipid antibodies (APLA) in this patient population is rare. Following treatment with six cycles of bendamustine and rituximab with 2 years of rituximab maintenance, our patient remained in remission and his coagulation studies normalised. This report describes a case of successful treatment of APLA associated with MZL that resolved after treatment of the lymphoma.


Assuntos
Anticorpos Antifosfolipídeos/efeitos dos fármacos , Síndrome Antifosfolipídica/tratamento farmacológico , Cloridrato de Bendamustina/uso terapêutico , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Rituximab/uso terapêutico , Anticorpos Antifosfolipídeos/sangue , Antineoplásicos Alquilantes/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Síndrome Antifosfolipídica/complicações , Cloridrato de Bendamustina/administração & dosagem , Diagnóstico Diferencial , Humanos , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/patologia , Linfoma de Zona Marginal Tipo Células B/patologia , Masculino , Pessoa de Meia-Idade , Doenças Raras , Rituximab/administração & dosagem , Resultado do Tratamento
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