Monoklonální imunoglobuliny se v ojedinělých případech váží na tělu vlastní antigeny a způsobují tak autoimunitní poškození organizmu. V textu popisujeme Waldenströmovu makroglobulinemii, která se manifestovala bolestmi ve svalech a anémií. Pacientka pociťovala bolesti zad asi 3 roky před příchodem na naši ambulanci. V posledním roce se k bolestem zádových svalů přidaly i bolesti ve svalech horních a dolních končetin. Vzniklo tak podezření na polymyozitidu. Ta ale nebyla odborným vyšetřením potvrzena. Pacientka měla v kostní dřeni jasnou infiltraci lymfoplazmocytárním lymfomem a 10,8 g/l monoklonálního imunoglobulinu typu IgM. Koncentrace myoglobinu v séru a aktivita kreatinkinázy (CK) v séru byly opakovaně signifikantně zvýšené. Proto byla zahájena léčba monoklonální protilátkou antiCD20 (Mabthera) 375 mg/m2 i. v. 1krát měsíčně, cyklofosfamidem 500 mg/m2 i. v. den 1 a den 15 v 28denním cyklu a dexametazonem 20 mg 1.–4. den a 15.–18. den léčebného cyklu. Plánováno bylo celkem 8 cyklů. Již po 5. cyklu bylo dosaženo vymizení monoklonálního imunoglobulinu (negativní imunofixace), normalizace hodnot myoglobinu a CK a výrazný ústup bolestí svalů. Koncentrace hemoglobinu před léčbou byla výrazně snížená, po léčbě se normalizovala. Po 5 cyklech bylo tedy dosaženo kompletní remise Waldenströmovy nemoci dle biochemických parametrů a normalizace hodnot myoglobinu a CK v séru., Waldenström‘s macroglobulinemia which was manifested by muscle pain and anemia. The female patient had suffered from back pain for about 3 years before she came to our clinic. In the last year pain in the muscles of the upper and lower extremities developed in addition to back pain. This led to the suspicion of polymyositis. However this was not confirmed by a special examination. The patient was diagnosed with clearly established infiltration of lymphoplasmacytic lymphoma and 10.8 g/l of type IgM monoclonal immunoglobulin in the bone marrow. Serum myoglobin levels and serum CK activity were repeatedly significantly increased. Therefore the treatment with anti-CD20 monoclonal antibody (Mabthera) 375 mg/m2 i. v. was started, administered once a month, with cyclophosphamide 500 mg/m2 i. v. on days 1 and 15 of a 28-day cycle, and dexamethasone 20 mg from 1st through to 4th days and 15th through to 18th days of the treatment cycle. There were 8 cycles planned. Already after a 5th cycle, the disappearance of monoclonal immunoglobulin (negative immunofixation), normalisation of myoglobin and CK values and significant relief from muscle pain were achieved. The hemoglobin concentrations before treatment were significantly reduced, while they were normalised after treatment. After 5 cycles, the complete remission of Waldenström‘s disease was reached according to biochemical parameters, and normalisation of the serum myoglobin and creatine kinase levels was achieved., and Zdeněk Adam, Jarmila Kissová, Luděk Pour, Marta Krejčí, Eva Ševčíková, Renata Koukalová, Zdeňka Čermáková, Marta Černá, Zdeněk Král, Jiří Mayer
Myopericytoma is a rare benign tumour composed of pericytic cells that show myoid differentiation and have a tendency for concentric perivascular growth. It belongs to a spectrum ofperivascular myoid cell neoplasms. To date, only a small number of cases of myopericytoma involving the oral cavity have been reported. We describe a case of myopericytoma presenting as a slowly growing tongue nodule in a 61-year-old woman. A diagnosis of myopericytoma was established with the histopathological findings combined with immunohistochemical staining. Myopericytoma should be included in the differential diagnosis of well-circumscribed, slow-growing lesions of the oral cavity. and S. Akbulut, D. Berk, MG. Demir, S. Kayahan
Myotropic neuropeptides were isolated from the retrocerebral complex of the stick insect, Carausius morosus, by using three HPLC steps. Bioactivity during purification was measured by heterologous bioassays monitoring the contractions of the hyperneural muscle and hindgut of the American cockroach. Additionally, fractions not active in these bioassays were tested in a homologous bioassay evoking contractions of the hindgut of C. morosus. Peptide sequence analysis and mass spectrometry yielded the following structures: Pro-Phe-Cys-Asn-Ala-Phe-Thr-Gly-Cys-NH2 (CCAP), pGlu-Thr-Phe-Gln-Tyr-Ser-His-Gly-Trp-Thr-Asn-NH2 (His7-corazonin) and Asp-Glu-Gly-Gly-Thr-Gln-Tyr-Thr-Pro-Arg-Leu-NH2 (Cam-PK-1). These neuropeptides are the first myotropins isolated from C. morosus. The most bioactive compound in the homologous bioassay, the C. morosus-hindgut assay, was CCAP., Reinhard Predel, Roland Kellner, Gerd Gäde, and Lit