Non-secretory multiple myeloma presenting as primary plasma cell leukaemia.

A case of non-secretory multiple myeloma presenting as primary plasma cell leukaemia in a 65 year old woman is presented. Bone pain was the initial clinical manifestation. Laboratory analysis showed 20% of circulating immature plasma cells. Despite the presence of osteolytic lesions, no M-component could be demonstrated in serum protein electrophoresis, and serum and urine immunoelectrophoresis. Bone marrow aspirate demonstrated an 83% infiltration of plasma cells showing various degrees of immaturity. Immunofluorescence with monoclonal antisera demonstrated intracytoplasmic kappa light chains in a high percentage of plasma cells. Immature plasma cells without cellular capacity to synthesize and excrete complete immunoglobulins could be more aggressive, leading to an initial leukaemic process. Previous work regarding possible pathogenetic mechanisms, clinical and laboratory features, and response to treatment of this extremely rare association are reviewed.     

Kaposi's sarcoma associated with hairy cell leukaemia

The case is described of a patient with hairy cell leukaemia who later developed Kaposi''s sarcoma. This association has not been reported before and the coexistence of hairy cell leukaemia with another tumour is very rare. The implications of this unusual association are briefly discussed.     

锁骨浆细胞骨髓瘤的影像特点分析

目的 总结锁骨浆细胞骨髓瘤的影像特征,提高对本病的影像认识水平。方法 分析本院2013 年3 月～2018 年12 月期间11 例锁骨肿瘤性病变并经手术病理诊断为浆细胞骨髓瘤的影像表现,其中X 线、CT 及MRI均检查6 例,X 线和CT 检查4 例,单行CT 检查1 例。结果 X线表现膨胀性改变明显8 例,膨胀性改变不明显2 例,骨皮质变薄、中断6 例。11 例CT 表现均有不同程度的膨胀性改变及骨小梁破坏、消失,其中5 例病灶中骨嵴形成,8 例软组织肿胀明显,伴肿块形成。6 例MRI 检查中有2 例病灶信号混杂,边界不清,4 例呈长T1 长T2 信号影,脂肪抑制像上条片状较T2 更高信号影。结论 锁骨浆细胞骨髓瘤在X 线、CT 及MRI 分别有不同的影像特征;三种检查方法联合使用能全面展示浆细胞骨髓瘤的影像特征,更有利于提高对该病的认识。     

Survival of twenty-two months in a patient with primary plasma cell leukaemia treated with melphalan and prednisolone

In the majority of cases plasma cell leukaemia is a rapidly fatal disease with a mean survival time of five months. There have been reports of increased survival using various regimens of chemotherapy although most cases eventually relapse. We describe a patient with primary plasma cell leukaemia who responded to a combination of oral melphalan and prednisolone with control of the disease in the bone marrow but relapsed with extramedullary disease in the central nervous system and testes, and died 22 months after diagnosis. Melphalan poorly penetrates the central nervous system and its testicular penetration is unknown.     

孤立性浆细胞性骨髓瘤影像诊断1例

孤立性浆细胞瘤是一种少见的骨髓瘤，约占骨髓瘤10％，术前诊断困难。我院遇到1例，现报告如下。     

Primary malignant neoplasms associated with chronic lymphocytic leukaemia

The relationship between chronic lymphocytic leukaemia (CLL) and primary malignant neoplasms was evaluated using data from the Hematology Division in Beilinson Medical Center and the Israel Cancer Registry. The study population consisted of 81 patients diagnosed between 1962 and 1984. A total of 16 patients were found to have 21 malignant neoplasms in addition to their CLL. Excluding patients with nonmelanoma skin tumours, a 1.7 increased risk (statistically not significant) for developing second malignant neoplasms in CLL patients was detected. The only tumour which occurred significantly more than expected subsequent to CLL diagnosis was brain cancer. The coexistence of multiple cancers in the same patient was diagnosed in four of the patients. The results of this study further support the hypothesis that patients with CLL are prone to develop second neoplasms.     

骨髓组织印片在浆细胞骨髓瘤形态学诊断中的价值

目的 了解骨髓组织印片在浆细胞骨髓瘤诊断中的应用价值.方法 收集同步进行骨髓涂片、组织印片和切片检查的98例浆细胞骨髓瘤,比较骨髓涂片、组织印片和切片在有核细胞量、浆细胞百分比、浸润度及诊断符合率的差异.结果 骨髓组织印片显示有核细胞量评估性能优于涂片（P＜0.05）,而与切片相近（P＞0.05）;骨髓组织印片显示浆细胞含量高于涂片（P＜0.05）;骨髓组织印片显示浸润度优于涂片（P＜0.05）,而与切片相近（P＞0.05）;骨髓涂片由于受稀释或干抽等因素影响符合率仅为83.7％,而骨髓组织印片诊断符合率高达98.9％,骨髓组织印片诊断符合率优于骨髓涂片（P＜0.05）.结论 骨髓组织印片兼有涂片和切片的特征;组织印片联合涂片互补性检查,对浆细胞骨髓瘤形态学诊断具有明显优势.     

Type-B lactic acidosis associated with progressive multiple myeloma

We report a 64-year-old lady with stage II, Immunoglobulin-G lambda multiple myeloma (MM) (standard risk), who presented with type-B lactic acidosis (LA), and multi-organ dysfunction associating myeloma progression, and ending in imminent death. In the context of literature review of all previously reported similar cases, this report highlights and discusses the association of type-B LA and MM (especially progressive disease), and also emphasizes the poor outcome. Early recognition of this condition with intensive supportive care, and treatment of multiple myeloma may improve outcomes.Metabolic acidosis (MA) can be defined as decreased systemic pH resulting from either an increase in hydrogen ion (H⁺), or a reduction in bicarbonate (HCO₃^-). Based on the etiology, it is classified into anion gap MA and non-gap MA. Lactic acidosis (LA) is a common cause of the high anion gap MA.1 It is the normal end-product of the anaerobic breakdown of glucose in the cells.

Pyruvate+NADH+H+↔lactate+NAD+

Also the normal level of lactic acid in the serum is 0.5-1 mmol/l.1 Lactic acid can accumulate in the blood due to increased production or decreased utilization. Lactic acidosis occurs when there is an increase in lactate levels (>4 mmol/l) along with MA.1 This results in several adverse effects on human physiology. Lactic acidosis can be classified based on the pathogenesis into 2 categories: type-A LA and type-B LA.1 Type-A LA is diagnosed when there is clinical evidence of decreased tissue perfusion, or oxygenation of blood and it results from either: 1) increased production of lactate; such as in the cases of hypovolemia, cardiac failure, sepsis, and cardiopulmonary arrest, or 2) from the diminished utilization of lactate, such as in liver disease, and in thiamine deficiency.1 In contrast, type-B LA occurs when there is no evidence of decreased tissue perfusion, or oxygenation. This can occur with systemic diseases, such as renal and hepatic failure, diabetes mellitus, malignancy, toxins, drugs, and inborn errors of lactate metabolism. The link between type-B LA and malignancy is established, especially in leukemia and lymphoma.2 This case report describes the association of multiple myeloma (MM) with type-B LA, and highlights the prognostic impact of this association in a background of literature review of all reported cases of MM and LA.     

Pseudoerythrocytosis in myeloma with associated peripheral neuropathy.

A number of unusual features have been described in patients who have peripheral neuropathy in association with plasma cell neoplasia. Amongst these features are raised haemoglobins and increased platelet counts. We have measured the red cell mass in one such patient and have shown that the raised haemoglobin was due to a pseudoerythrocytosis. Our patient is also of interest because of his youth being only 19 years old at presentation.