多发性骨髓瘤继发浆细胞白血病一例

<正>1病例介绍患者,女,45岁,2019年2月因“持续胸背痛伴发热”就诊于福建省立医院血液科,血常规检查示:白细胞8.5×10⁹/L,浆细胞14%,血红蛋白66 g/L,血小板150×10⁹/L。生化:白蛋白31 g/L,球蛋白77 g/L,肌酐143μmol/L,钙离子2.69 mmol/L,磷1.68 mmol/L,乳酸脱氢酶216 U/L。24 h尿蛋白0.534 g/24 h。β₂微球蛋白(血) 6.21 mg/L。血:κ轻链22.70 g/L,λ轻链0.08 g/L,κ/λ283.75。尿:κ轻链255.00 mg/L,λ轻链<3.81 mg/L。     

门静脉闭锁致门脉高压相关性肺动脉高压1例并文献复习

目的:复习罕见门静脉解剖异常导致门脉高压性肺动脉高压(PPHTN)的相关文献,了解这类患者临床特点。方法:回顾分析1例成人门静脉闭锁导致PPHTN患者的临床经过和特点,并以"门静脉闭锁"、"门脉高压性肺动脉高压"为检索词,在万方数据库和中国全文期刊数据库中进行检索,以"atresia of portal vein"、"pulmonary hypertension"为检索词在pubmed全文数据库中进行检索。结果:患者女性,71岁,3年前因反复"肝性脑病"发现门静脉主干闭锁,脾静脉纤细;肠系膜上静脉-左肾静脉门体分流形成。胃底周围见静脉曲张,门腔静脉自发分流形成可能。2年6个月前出现双下肢水肿,1月前活动耐力明显下降,伴夜间阵发性呼吸困难,伴腹胀。UCG示下腔静脉增宽,心室呈"D"型影,s PAP84mm Hg(1mm Hg=0.133kPa)。因患者入院后出现院内感染及急性肾损伤,未能行右心导管检查,结合病史及检查结果,除外肺实质疾病、肺血栓栓塞症、结缔组织疾病及其他可能导致肺动脉高压(PAH)的药物使用或毒物接触史,同时确定患者存在门脉高压导致的胃底静脉曲张,诊断患者为非肝病性门静脉解剖异常导致的PPHTN。共检索中文文献2篇,外文文献24篇,保留较为详细病例资料文献15篇,涉及病例25例。结论:非肝病性门脉高压所形成的门体分流可以导致高动力性PAH,先天性门体分流者多见,继发于成人门静脉闭锁者罕见,早期识别随访,及时针对PAH进行干预治疗,维护右心功能,可能会延迟右心衰竭发生的时间,改善预后。     

系统性红斑狼疮并发肺动脉高压1例并文献复习

目的提高对系统性红斑狼疮（SLE）并发肺动脉高压（PHT）的发病机制、临床表现、治疗及预后的认识。方法对1例并发PHT的SLE病例进行分析和讨论,并结合相关文献复习。结果SLE并发PHT的机制尚不明确。其临床表现无特异性,早期症状多为呼吸困难。SLE患者出现雷诺征时,应高度怀疑PHT的存在。大剂量糖皮质激素联合免疫抑制剂可显著降低PHT。结论SLE并发PHT常提示预后不良,应尽早诊断及治疗。针对原发病的强化治疗可有效降低PHT。     

多发性骨髓瘤合并慢性中性粒细胞白血病一例并文献复习

目的 探讨多发性骨髓瘤（MM）合并慢性中性粒细胞白血病（CNL）的主要临床特点、诊断和治疗方法.方法 分析我科收治的1例MM合并CNL患者的临床表现及相关实验室检查,总结国内外报道的共29例患者的主要特征,并查阅相关文献,探讨本病的治疗方法.结果 MM合并CNL是一种罕见疾病,可能源于共同的造血干细胞异常,我们采用CTD方案化疗4个疗程,取得满意效果.结论 MM合并CNL罕见,其病因、发病机制及治疗方案需进一步探讨,CTD方案可能是有效的治疗本病的方法.     

以肺动脉高压为首发症状的系统性红斑狼疮1例报告并文献复习

<正> 1 病例介绍患者,女,42岁。因"活动时气急三月余"于2009-02-25入我院,3个月前开始出现活动时气急,且伴有胸闷,常感倦怠、乏力,但休息5～6 min后病情可自行缓解,当时患者未给予重视,未经诊治,后病情有所发展,且夜间无明显诱因下出现胸闷、气急,遂于2009-02-24来我院就诊,拟"肺动脉高压"收住心内科,后转血液风湿科。患者发病以来除经常"感冒"外,无畏寒、发热、咳嗽、咳痰,无头晕、头痛、恶心、呕吐、胸痛,有脱发、雷诺现象。否认高血压、糖尿病、肝炎、结核史,否认遗传性家族疾病史。入院查体:神志清楚,自主体位,全身皮肤黏膜无黄染、瘀点、瘀斑,浅表淋巴结未及肿大,咽无充血,扁桃体无肿大,两肺呼吸音粗糙,左下肺可闻及少许湿啰音,心率90次/min,律整,肺动脉瓣区第二心音亢进伴分裂,余瓣膜区未闻及杂音,腹平软,肝脾肋下未触及,移动性     

浆细胞白血病与多发性骨髓瘤的临床及细胞形态学观察对比

从临床和实验室两个方面观察分析了浆细胞白血病和多发性骨髓瘤的异同点:两者均有骨髓中浆细胞恶性增生、骨痛等,但原发性浆细胞白血病起病急,病程短,细胞形态偏于幼稚,疗效差;多发性骨髓瘤则骨损害严重,化疗反应相对好,病程相对长,当其外周血浆细胞≥20%,即为继发性浆细胞白血病。     

以肺动脉高压为主要表现的系统性红斑狼疮一例

患者，女，34岁，已婚，农民。因活动时气促4年，加重1年于2004年2月17日入院。患者4年前元明显诱因逐渐出现活动时气促，经休息可缓解，体力明显下降。在家未作治疗，患者气促呈逐渐加重趋势，特别是近1年来气促明显加重，稍活动即感气促，并出现下午双下肢浮肿，脱发，反复咳嗽、咳少许白色黏液样痰。无发热、皮疹、关节肿痛，无少尿及肉眼血尿，无口腔溃疡。门诊做心脏B超检查后以“心脏扩大原因待查”收入我院心内科。     

原发性浆细胞白血病1例

患者女性 ,6 9岁。因左上臂疼痛 ,发热 1个月在院外治疗效果不佳 ,于 2 0 0 1年 5月 6日来我院诊治。体检 :ＢＰ180 /10 0ｍｍＨｇ ,重病面容 ,神清 ,浅表淋巴结不大 ,胸骨叩击痛 (+)。心率 10 0次 /ｍｉｎ ,律齐 ,无杂音 ,双下肺可闻及少许湿罗音。左上臂呈局限性水肿 ,有触痛。腹软 ,肝脾不大。血常规 :白细胞 (30～ 5 3)× 10 9/Ｌ ,红细胞 (1.85～ 2 .42 )×10 12 /Ｌ ,血红蛋白 6 8～ 79ｇ/Ｌ。血小板 (16 5～ 32 5 )× 10 9/Ｌ。肝功能ＴＰ 10 0 ｇ/Ｌ ,ＡＬＢ 2 5 ｇ/Ｌ ,Ａ/Ｇ0 .3,ＧＰＴ正常。尿本周氏蛋白 (一次 )阴性。ＩｇＧ16 …     

原发性肺动脉肉瘤1例报告并文献复习

原发性肺动脉肉瘤是起源于动脉内膜并且少见的恶性肿瘤,经常难以与肺动脉栓塞鉴别,给准确诊断与治疗带来困难.我们报道了一例肺动脉肉瘤患者并复习相关文献,旨在提高对肺动脉肿瘤认识及诊断治疗水平.     

以肺动脉高压为主的混合性结缔组织病报告并文献复习

目的:探讨混合性结缔组织病并发肺动脉高压的诊断与治疗,从而提高对该病的认识.方法:报道1例以肺动脉高压为首诊的混合性结缔组织病临床资料并复习国内相关报道.结果:国内之前报道8例,包括本例共9例,全为女性;首发主要症状是胸闷、心悸、气喘;临床表现为雷诺现象、关节肿胀及疼痛、硬指/手指肿胀、紫绀等;辅助检查发现血沉升高、血...     

硼替佐米治疗继发性浆细胞白血病1例并文献复习

目的:探讨硼替佐米治疗浆细胞白血病的疗效.方法:报道1例继发性浆细胞白血病患者应用硼替佐米治疗的疗效,对硼替佐米治疗浆细胞白血病的相关文献进行复习.结果:该例患者经包括自体造血干细胞移植在内的多种治疗,二次复发并进展为继发性浆细胞白血病,经硼替佐米为基础的方案治疗后获得满意的近期疗效,文献复习显示硼替佐米治疗浆细胞白血...     

多发性骨髓瘤伴胸膜浸润1例

1病历资料患者女,77岁。2010年2月起反复出现胸闷、气急,无发热、咳嗽、盗汗、胸痛、心悸等不适,夜间尚可平卧。既往有高血压病史20余年,血压最高达180/110 mmHg(1 mmHg=0.133 kPa),平素服用降压药,血压控制良好。入院体检:神志清楚,消瘦,全身皮肤无黄染,无皮下出血点,球结膜无水肿,颈     

Primary nonsecretory plasma cell leukemia: a rare variant of multiple myeloma

 We report a patient with primary nonsecretory plasma cell leukemia. These cells resembled prolymphocytes and expressed only CD38 and CD56. Due to the atypical morphology and lack of monoclonal immunoglobulins in both serum and urine, it was difficult to make a correct diagnosis. Electron-microscopic and immunocytochemical cytoplasmic studies were useful. Received: May 4, 1998 / Accepted: September 3, 1998     

Pulmonary vein stenosis: Case report and literature review

BACKGROUND:

Pulmonary vein stenosis is a rare cause of pulmonary hypertension, with variable onset and presentation. One or more of the four pulmonary veins can be primarily or secondarily affected. A five-month-old girl presented with respiratory distress, lethargy and cyanosis requiring intubation.

METHODS:

Echocardiography showed right ventricular dilation, a right ventricular systolic pressure of 97 mmHg, decreased ejection fraction and turbulent flow at the left atrium. Cardiac catheterization revealed stenosis of the left-sided pulmonary veins, for which she underwent a Coles procedure.

RESULTS:

Postoperatively, there was a transient improvement in the patient’s pulmonary hypertension but she subsequently deteriorated. Her prognosis was considered bleak, and a decision was made with the family to withdraw care.

CONCLUSION:

Pulmonary vein stenosis is a rare cause of pulmonary hypertension, and is associated with significant morbidity and mortality. Surgical intervention may be of benefit in selected cases. It stands to reason that any treatment will have the best chance of success if completed before the pulmonary hypertension becomes fixed. Early diagnosis depends on a high index of clinical suspicion.     

Primary plasma cell leukemia with extensive dense osteosclerosis: complete remission following combination chemotherapy

Diffuse osteosclerotic myeloma is very rare, and primary plasma cell leukemia with extensive osteosclerosis is even more rare. We describe a 71-year-old man who presented with severe anemia and dense widespread osteosclerosis similar to the X-ray finding of myelosclerosis. His peripheral blood showed 40% plasma cells. Bone marrow examination revealed heavy plasma cell infiltration with marked myelofibrosis and myelosclerosis. Protein electrophoresis and immunoelectrophoresis demonstrated an M-protein of IgG-lambda type. He was treated with cyclophosphamide, vincristine, and prednisolone for 10 months. A complete remission was obtained, with disappearance of M-protein and circulating plasma cells and normalization of complete blood counts, bone marrow picture, and biochemical parameters, as well as complete regression of myelofibrosis and osteosclerotic lesions. Unmaintained complete remission lasted for more than 1 year and he survived for more than 22 months. Our case indicated that one must include in the differential diagnosis of an osteosclerotic lesion the possibility of multiple myeloma, and that combination chemotherapy can induce a complete remission in this disease.     

肺母细胞瘤1例并文献复习

目的提高对肺母细胞瘤的认识。方法结合1例肺母细胞瘤患者的临床资料和文献复习,详细分析该病的组织起源、临床病理特点、分类、诊断、鉴别诊断、治疗及预后等。结果该病临床症状少且轻微,影像学提示“良性肿瘤”;病理学检查主要特征是镜下瘤组织由胎儿型腺管样的上皮成分和原始的肉瘤样间质成分混杂存在。免疫组化示瘤组织由上皮和间叶两种成分构成。目前尚无有效治疗措施,预后差。结论肺母细胞瘤罕见,极易误诊,应予重视。     

Cytogenetics in multiple myeloma and plasma cell leukemia: simultaneous cytogenetic and cytologic studies in 51 patients

Summary Cytogenetic studies in patients with multiple myeloma (MM) and plasma cell leukemia (PCL) have in general been largely unsuccessful. The investigation of mitoses of nonmalignant hematopoietic precursor cells, rather than mitoses of malignant plasma cells might account for the low percentage of pathological genetic findings. We investigated bone marrow (BM) cells of 51 patients both cytogenetically and cytologically. In patients with a normal karyotype (n=39) nearly all mitoses examined cytologically (107/117) derived from granulopoietic or erythropoietic cell lineages. In contrast, 20/27 metaphases in patients with a pathological karyotype (n=12) were found to be plasma cell mitoses. These findings may explain the low rate of chromosomal rearrangements in MM and may suggest that the real abnormality rate is considerably higher.     

不典型毛细胞白血病1例报告并文献复习

目的:探讨毛细胞白血病(HCL)不典型特征的鉴别思路.方法:报告1例不典型HCL的临床特征、诊断、治疗及预后情况,简述HCL诊断的最新进展.结果:结合患者临床特征和各项辅助检查结果,对不典型特征进行进一步鉴别,确诊为HCL.结论:HCL诊断时应结合患者临床特征和辅助检查,遇不典型特征应作进一步鉴别以防出现误诊、漏诊.     

Gastrointestinal bleeding as initial presentation of extramedullary plasma cell neoplasms: A case report and review of the literature

BACKGROUND Plasma-cell neoplasms rarely involve the gastrointestinal tract and manifest as gastrointestinal bleeding. Plasmablastic myeloma is an aggressive plasma cell neoplasm associated with poor outcomes. A small number of cases with gastrointestinal involvement is reported in the literature and therefore high index of suspicion is essential for avoiding delays in diagnosis and treatment.CASE SUMMARY Our aim is to present our experience of a 70-year-old patient with a secondary presentation of plasmablastic myeloma manifesting as unstable upper gastrointestinal bleeding and to review the literature with the view to consolidate and discuss information about diagnosis and management of this rare entity. In addition to our case, a literature search(Pub Med database) of case reports of extramedullary plasma cell neoplasms manifesting as upper gastrointestinal bleeding was performed. Twenty-seven cases of extramedullary plasmacytoma(EMP) involving the stomach and small bowel presenting with upper gastrointestinal bleeding were retrieved. The majority of patients were males(67%). The average age on diagnosis was 62.7 years. The most common site of presentation was the stomach(41%), followed by the duodenum(15%). The most common presenting complaint was melena(44%). In the majority of cases, the EMPs were a secondary manifestation(63%) at the background of multiple myeloma(26%), plasmablastic myeloma(7%) or high-grade plasma cell myeloma(4%). Oesophagogastroscopy was the main diagnostic modality and chemotherapy the preferred treatment option for secondary EMPs.CONCLUSION Despite their rare presentation, upper gastrointestinal EMPs should be considered in the differential diagnosis of patients with gastrointestinal bleeding especially in the presence of systemic haematological malignancy.