Adam Z, Bednarík J, Neubauer J, Chaloupka R, Fojtík Z, Vanícek J, Pour L, Cermákova Z, Scudla V, Maisnar V, Straub J, Schützová M, Gregora E, Weinreb M, Stuchlíková K, Stanícek J, Hájek R, Krejcí M, Vorlícek J
Interní hematoonkologická klinika Lékarské fakulty MU a FN Brno.
Vnitr Lek. 2006 Nov;52 Suppl 2:9, 11-31.
The number of newly diagnosed cases of multiple myeloma in the Czech Republic is about 3-4 per 100 000 persons per year. In the higher age groups, the incidence increases. Multiple myeloma is an illness that reacts well to treatment which can result in periods of remission lasting for years. Some of the patients are even able to return to work. A pre-requisite for successful treatment is early diagnosis and this is usually in the hands of first line physicians. This is the reason why the Czech Myeloma Group, in conjunction with neurologists, orthopedicians and radio diagnosticians has issued the following recommendations for first line physicians containing a more detailed description of the symptoms and the diagnostic pitfalls of the disease. This disease reminds a chameleon for the variety of its symptoms. For the sake of clarification, we shall divide multiple myeloma symptoms into five points, each of which is reason enough to warrant an examination to confirm or rule out a malignant cause of health problems (a negative result does not automatically mean exclusion). If any of the recommended examinations results positive, the diagnostic process must be continued, in which case a general practitioner refers the patient to a specialist health centre. Observing these recommendations should minimize the number of cases of late diagnosis. 1. Bone destruction symptoms. - Unexplained backache for more than one month in any part of spine even without nerve root irritability or without pain in other part of skeleton (ribs, hips, or long bones). - Pain at the beginning of myeloma disease is very similar to benigne common discopathy, however the intensity of backache is decreasing within one months in benigne disease. In the case of malignant process the intensity of bone pain is steadily increasing. - Immediate imaging and laboratory investigation are indicated by resting and night pain in spinal column or in any part of skeleton. - Backache with the sign of spinal cord or nerve compression should be sent for immediate X Ray, and focussed CT/MRI followed by acute surgery if needed. - Osteoporosis especially in men and premenopausal women. 2. Features of changed immunity or bone marrow function. Persistent and recurrent infection, typical is normochromic anaemia, with leucopenia and trombocytopenia. 3. Raised erythrocyte sedimentation rate even increase concentration of total plasma protein. 4. Impaired renal function. Increased level of creatinin or proteinuria, nephrotic syndrome with bilateral legs oedema. 5. Hypercalcemia with typical clinical symptoms (polyuria with dehydratation, constipation, nausea, low level conscience, coma). Every one from these points has to be reason for general medical doctor to start battery of tests: -X-ray of bones focused to painful area (mandatory before physiotherapy, local anaesthesia or other empiric therapy). If plain X-ray does not elucidate pain and symptoms are lasting more than one month, please consider all circumstances and results from laboratory investigation. This patient needs referral to the centre with MRI/CT facilities (CT or MRI is necessary investigation in case of nerve root or spine compression). -Investigation of erythrocyte sedimantion rate (high level of sedimentation of erythrocyte can indicate multiple myeloma). -Full blood count. -Basic biochemical investigation serum and urine: serum urea, creatinin, ionts including calcium, total protein, and albumin CRP (high concentration of total protein indicates myeloma, low level of albumin indicates general pathological process, similary increased concentration of fibrinogen, impaired renal function indicates myeloma kidney, however hypercalcemia is typical for highly aggressive myeloma). -Quantitative screening for IgG, IgM and IgA in serum (isolated raised level one of immunoglobulin with decreased level of the others indicates myeloma). -Common electrophoresis of serum is able to detect monoclonal immunoglobulin level at few gramm concentration. If all the laboratory investigation are in normal level the possibility that the current problems are multiple myeloma origine is smaller, but it does not exclude one of rare variant--non secretory myeloma (undifferentiated plasmocyt lost characteristic feature to produce monoclonal immunoglobulin). If any of tests indicate the possibility of myeloma, patient require urgent specialist referral to department with possibility to make diagnosis of malignant myeloma.
捷克共和国每年每10万人中新诊断出的多发性骨髓瘤病例数约为3 - 4例。在较高年龄组中，发病率会上升。多发性骨髓瘤是一种对治疗反应良好的疾病，治疗后可能会有持续数年的缓解期。有些患者甚至能够重返工作岗位。成功治疗的一个先决条件是早期诊断，而这通常掌握在一线医生手中。这就是为什么捷克骨髓瘤小组与神经科医生、骨科医生和放射诊断医生联合为一线医生发布了以下建议，其中包含了对该疾病症状和诊断陷阱的更详细描述。这种疾病因其症状多样，就像一条变色龙。为了便于说明，我们将多发性骨髓瘤的症状分为五点，每一点都足以成为进行检查以确认或排除健康问题的恶性病因的理由（阴性结果并不一定意味着排除）。如果任何一项推荐检查结果呈阳性，诊断过程必须继续，在这种情况下，全科医生会将患者转诊至专科健康中心。遵循这些建议应能减少晚期诊断的病例数。1. 骨破坏症状。 - 脊柱任何部位不明原因的背痛持续超过一个月，即使没有神经根刺激症状或骨骼其他部位（肋骨、髋部或长骨）疼痛。 - 骨髓瘤疾病初期的疼痛与良性常见椎间盘病非常相似，但良性疾病中背痛强度在一个月内会减轻。而在恶性病变中，骨痛强度会持续增加。 - 脊柱或骨骼任何部位出现静息痛和夜间痛时，应立即进行影像学和实验室检查。 - 伴有脊髓或神经受压体征的背痛患者应立即进行X线检查，随后进行针对性的CT/MRI检查，如有需要则进行急诊手术。 - 骨质疏松，尤其是男性和绝经前女性。2. 免疫或骨髓功能改变的特征。持续和反复感染，典型表现为正细胞正色素性贫血，伴有白细胞减少和血小板减少。3. 红细胞沉降率升高，甚至总血浆蛋白浓度增加。4. 肾功能损害。肌酐水平升高或蛋白尿，伴有双侧腿部水肿的肾病综合征。5. 高钙血症及典型临床症状（多尿伴脱水、便秘、恶心、意识水平降低、昏迷）。这些要点中的每一点都必须成为全科医生开始进行一系列检查的理由： - 针对疼痛部位的骨骼X线检查（在物理治疗、局部麻醉或其他经验性治疗之前是必需的）。如果普通X线检查不能阐明疼痛情况且症状持续超过一个月，请综合考虑所有情况及实验室检查结果。该患者需要转诊至具备MRI/CT设备（在出现神经根或脊柱受压情况时，CT或MRI是必要检查）的中心。 - 红细胞沉降率检查（红细胞沉降率升高可能提示多发性骨髓瘤）。 - 全血细胞计数。 - 血清和尿液的基础生化检查：血清尿素、肌酐、包括钙在内的离子、总蛋白、白蛋白、CRP（总蛋白浓度升高提示骨髓瘤，白蛋白水平降低提示一般病理过程，类似地，纤维蛋白原浓度升高、肾功能损害提示骨髓瘤肾病，然而高钙血症是高度侵袭性骨髓瘤的典型表现）。 - 血清中IgG、IgM和IgA的定量筛查（一种免疫球蛋白单独升高而其他免疫球蛋白水平降低提示骨髓瘤）。 - 血清普通电泳能够检测出几克浓度的单克隆免疫球蛋白水平。如果所有实验室检查结果都在正常范围内，当前问题由多发性骨髓瘤引起的可能性较小，但并不排除罕见的变异型——非分泌型骨髓瘤（未分化浆细胞失去产生单克隆免疫球蛋白的特征）。如果任何一项检查提示有可能是骨髓瘤，患者需要紧急转诊至能够诊断恶性骨髓瘤的专科科室。
