低蛋白血症で発見された骨髄腫関連疾患の一症例

広義の骨髄腫関連疾患には,古典的なmyelomaを始めとして,plasmacytic leukemia,plasmacytomaさらにmacroglobulinemia,lymphoplasmacytic lymphomaなどが含まれる.一般的には特徴的検査所見の一つに,血清総蛋白の高値がある.ところが,今回我々は血清総蛋白がむしろ低値であった骨髄腫関連疾患を経験した.症例は85歳女性.貧血軽度,黄疸(-),肝脾腫(-),リンパ節腫大(-),骨病変は軽微であった.検査所見:末梢血WBC4370/μl,RBC242万/μl,Hgb8.1g/dl,Hct25.6%,PLT6.2万/μl,Neut70.7%,Lym23.6%,Mono5.0%,Eos0.2%,Bas0.5%.生化学:TP5.3g/dl,Alb2.0g/dl,Glob3.3g/dlそのうちIgG532mg/dl,IgA79mg/dl,IgM2,035mg/dlで,免疫電気泳動にて明らかなM-bowを認め,IgM-_K型と判明した.骨髄:採取した標本では低形成で,Mgk10/μl,赤芽球15.6%,顆粒球系60.3%,リンパ球系23.8%,そのうち形質細胞1.9%であった.形態学的には,マクログロブリン血症の際にみられるリンパ・形質細胞様の所見であった.細胞表面マーカーの検索では,リンパ球全体ではCD7 33.5%,CD138 0.7%,CD19 7.0%,CD20 32.8%,形質細胞ではCD7 21.8%,CD138 57.7%,CD19 21.1%,CD20 39.7%であった.病理検査では,N/C比が高く異型性のある核を持つ細胞のシート状の集簇が認められた.腫瘍細胞の透過型電顕所見では,核は偏在し,大型のゴルジ野を有し,粗面小胞体はよく発達し,蛋白合成の盛んなことが推測された.細胞によっては,分化度が低く核クロマチンは繊細で明らかに芽球様の細胞も認められた.染色体分析:46,XX.末梢血生化学所見では総蛋白量の明らかな減少が認められたが,その病因は腫瘍細胞によるIgMの過剰産生にあり,その腫瘍性性質のため正常免疫グロブリン特にIgGおよびIgAの著しい産生抑制を生じたものと考えられる.細胞学的にはマクログロブリン血症と多発性骨髄腫とにまたがる境界領域に位置付けられるBリンパ球系悪性疾患と推定される.It is known that myeloma-related disorders include classical myeloma, plasmacytic leukemia, plasmacytoma, macroglobulinemia, lymphoplasmacytic lymphoma and others. Increased serum protein is one of the characteristic features in these disorders. We have recently experienced a case of myeloma-related disorder with hypoproteinemia. The patient (a 85 year-old female) demonstrated hypoproteinemia (total protein 5.3g/dl, Alb2.0g/dl, Glob3.3g/dl) with increased IgM 2,035mg/dl contrary to decreased IgG(532mg/dl) and IgA(79mg/dl). The presence of IgM-_K monoclonality was detected by immunoelectrophoresis. Peripheral blood showed slight anemia(RBC 2.42×10^6/μl) and thrombocytopenia(62×10^3/μl). Peripheral lymphocytes were 23.6% with lymphoplasmacytic appearance. Bone marrow examination revealed that lymphocytes were 23.8% with 1.9% of plasma cells: CD79a(+), CD138(+/-), CD20(+/-), CD5(-), cyclin D1(-), IgM(+), IgA(-), IgG(+-), and IgM_K -monoclonality(+) by immune staining method. Surface makers of all lymphoid cells indicated CD138 57.7%, CD19 21.1%, and CD20 39.7%. Clusters of malignant lymphoid cells were present in bone marrow specimen. Transmission electron microscopy (TEM) on the lymphoid cells indicated the dislocated nucleus and profound rough-surfaced ribosomes with large Golgi apparatus suggesting the markedly enhanced protein production. In some cells, the blastic appearance with fine nuclear chromatin structure was observed. The pathogenesis of hypoproteinemia appears to be due to suppression of IgG and IgA by increased IgM monoclonality, in addition to decreased level of serum albumin. The disorder is considered to be localized at the border area between classical myeloma and macroglobulinemia

Thyroid Ultrasound Findings in Children from Three Japanese Prefectures: Aomori, Yamanashi and Nagasaki

Due to the likelihood of physical and mental health impacts following the unprecedented accident at the Fukushima Daiichi Nuclear Power Plant, the Fukushima prefectural government decided to conduct the Fukushima Health Management Survey to assist in the long-term health management of residents. This included thyroid ultrasound examination for all children in Fukushima. For appropriate evaluation of ultrasound screening of the thyroid, it is important to understand its reference data of thyroid findings in children in general. In order to analyze the frequencies of specific thyroid findings, we conducted ultrasound screening of the thyroid by the same procedures as used in Fukushima in 4,365 children, aged 3 to 18 years, from three Japanese prefectures. Overall, thyroid cysts were identified in 56.88% and thyroid nodules in 1.65% of the participants. Thyroid cysts and nodules with a maximum diameter of more than 5 mm were identified in 4.58% and 1.01%, respectively, and age-adjusted prevalences were 3.82% and 0.99%, respectively. Although the prevalence of cysts and nodules varied among the examination areas, no significant differences were observed among the three examination areas in the prevalence of cysts and nodules with a maximum diameter of more than 5 mm. Also, the prevalence of thyroid cysts and nodules, especially those with a maximum diameter of more than 5 mm, significantly increased with age, and showed a female predominance. We also identified ectopic thymus (1.95%), diffuse goiter (1.40%), ultimobranchial body (0.73%), lymph node swelling (0.21%) and thyroid agenesis (0.05%). This is the first ultrasound description of the age-adjusted prevalence of thyroid cysts and nodules, or of the prevalence of abnormalities other than cysts and nodules, such as ectopic thymus, in relation to age, in the general Japanese child population. We contend that this can provide relevant information for the Fukushima Health Management Survey and future population studies