Unexpected hope for a multiple myeloma patient

Multiple myeloma (MM) is a plasma cell neoplasm, characterized by periods of remission and relapses. The emergence of novel therapies, with multiple mechanisms of action and fewer adverse reactions, brings more and better options and also a higher survival rate. However, MM is still an incurable disease, and patients eventually become refractory to an extensive range of therapies. We present the case of a patient diagnosed with MM standard risk, who was at first refractory to multiple treatment regimens, and then had an unexpected and stable complete response to a newer drug of the same class

Applications of Corticosteroid Therapy in Inflammatory Rheumatic Diseases

Corticosteroids still remain the anchor drugs in therapy strategies for patients with inflammatory rheumatic diseases even though new drugs such as biologic or targeted synthetic molecules have emerged in the past years, being the most commonly prescribed medicines in the world due to their powerful immune-modulating properties. In this chapter, we aim to discuss the main characteristics of the glucocorticoids, their mechanism of action and effects on the immune system given the fact that they reduce the activation, proliferation, differentiation and survival of inflammatory cells such as macrophages and lymphocytes. Nevertheless, of great importance are the indications and tapering regimens, but also the adverse effects and various methods of monitoring the corticosteroid therapy

LEUKEMIJA I TRUDNOĆA. NIJE DALJE ŠTETNA POVEZANOST?

Purpose. Even though there are no solid data regarding chemotherapy treated leukemia during pregnancy, the results based on short series reports show that the management of such condition can be safely achieved during the second and third trimester. We present three personal cases of pregnant women treated with cytostatic agents, two of them accidentally receiving complete chemotherapy during the entire pregnancy without malformative consequences. First case. A 19 yrs old woman diagnosed with chronic myeloid leukemia who conceived spontaneously and mistook the pregnancy signs for a relapse of the disease. During the pregnancy she continued the treatment, receiving until the fifth month an association of Hydroxyurea and alfa-interferon and afterwards switched to Imatinib until term. She presented at 38–39 weeks and delivered by cesarean section a little girl of 3510 g in a perfect state of health. The blood count of both mother and child were normal. Second case. A similar situation in a young woman with lymphoblastic acute leukemia under treatment with Vincristin, Methotrexat, Purinethol. She presented in advanced spontaneous labour at 33–34 weeks and delivered a little girl of 1700 g without malformative signs and normal blood count. Third case. A 17 years old girl who was diagnosed with acute myeloid leukemia at 29 weeks pregnancy. She received induction chemotherapy with Ara-C, due to the significant bone marrow infiltrate and disease induced disseminated intravascular coagulopathy. She presented premature uterine contractions at 32 weeks and delivered by cesarean section a premature boy of 1750g with Apgar score 8. The infant did not present any malformation (by clinical and ultrasound examination) and the blood count was normal. The studies have shown so far that in the case of chronic myeloid leukemia, the treatment with Imatinib was associated with 50% apparently normal live infants and that chemotherapy for acute leukemia during the second or third trimester may not require termination of pregnancy, because both remission and delivery of a normal infant are likely to be obtained.SAŽETAK. Cilj. Uopće nema čvrstih podataka o kemoterapijom liječenim leukemijama tijekom trudnoće. Rezultati na temelju kratkih izvješća pokazuju da liječenje tijekom drugog i trećeg tromjesečja može biti uspješno obavljeno. Prikazujemo tri trudnice liječene citostaticima, dvije od njih su bez posljedičnih malformacija primale kompletnu kemoterapiju tijekom cijele trudnoće. Prvi slučaj. Žena od 19 godina koja je spontano zanijela i krivo shvatila znakove trudnoće kao recidiv bolesti. Tijekom trudnoće je nastavila liječenjem, primivši do petog mjeseca smjesu hidroksiureje i -interferona i zatim do termina imatinib. Javila se s 38–39 tjedana trudnoće te je carskim rezom rodila savršeno zdravu malu djevojčicu težine 3510 grama. Krvna slika majke i djeteta je bila potpuno normalna. Drugi slučaj. Sličan slučaj mlade žene s limfoblastičnom akutnom leukemijom, liječenom vinkristinom, metotreksatom, purinetolom. Javila se u uznapredovalom porodu s 33–34 tjedana te je rodila djevojčicu tešku 1700 grama, bez malformacija i s normalnom krvnom slikom. Treći slučaj. Djevojka od 17 godina kojoj je s 29 tjedana trudnoće dijagnosticirana akutna mijeloična leukemija. Primila je indukcijsku kemoterapiju Ara-C-om, zbog značajne infiltracije koštane srži te bolešću uzrokovane diseminirane intravaskularne koagulopatije. S 32 tjedna počeli su trudovi te je carskim rezom rodila nedonošena dječačića težine 1750 grama s Apgar zbrojem 8. Dijete nije imalo malformacija ni klinički niti ultrazvučnim pregledom. Krvna slika je bila normalna. Do sada su studije pokazale da kronična mijeloična leukemija, liječena imatinibom, u 50% slučajeva rezultira rađanjem zdrava djeteta te da kemoterapija akutne leukemije tijekom drugog i trećeg tromjesečja trudnoće na zahtijeva prekid trudnoće, jer se može postići remisija bolesti i rađanje normalna djeteta

LEUKEMIJA I TRUDNOĆA. NIJE DALJE ŠTETNA POVEZANOST?

Purpose. Even though there are no solid data regarding chemotherapy treated leukemia during pregnancy, the results based on short series reports show that the management of such condition can be safely achieved during the second and third trimester. We present three personal cases of pregnant women treated with cytostatic agents, two of them accidentally receiving complete chemotherapy during the entire pregnancy without malformative consequences. First case. A 19 yrs old woman diagnosed with chronic myeloid leukemia who conceived spontaneously and mistook the pregnancy signs for a relapse of the disease. During the pregnancy she continued the treatment, receiving until the fifth month an association of Hydroxyurea and alfa-interferon and afterwards switched to Imatinib until term. She presented at 38–39 weeks and delivered by cesarean section a little girl of 3510 g in a perfect state of health. The blood count of both mother and child were normal. Second case. A similar situation in a young woman with lymphoblastic acute leukemia under treatment with Vincristin, Methotrexat, Purinethol. She presented in advanced spontaneous labour at 33–34 weeks and delivered a little girl of 1700 g without malformative signs and normal blood count. Third case. A 17 years old girl who was diagnosed with acute myeloid leukemia at 29 weeks pregnancy. She received induction chemotherapy with Ara-C, due to the significant bone marrow infiltrate and disease induced disseminated intravascular coagulopathy. She presented premature uterine contractions at 32 weeks and delivered by cesarean section a premature boy of 1750g with Apgar score 8. The infant did not present any malformation (by clinical and ultrasound examination) and the blood count was normal. The studies have shown so far that in the case of chronic myeloid leukemia, the treatment with Imatinib was associated with 50% apparently normal live infants and that chemotherapy for acute leukemia during the second or third trimester may not require termination of pregnancy, because both remission and delivery of a normal infant are likely to be obtained.SAŽETAK. Cilj. Uopće nema čvrstih podataka o kemoterapijom liječenim leukemijama tijekom trudnoće. Rezultati na temelju kratkih izvješća pokazuju da liječenje tijekom drugog i trećeg tromjesečja može biti uspješno obavljeno. Prikazujemo tri trudnice liječene citostaticima, dvije od njih su bez posljedičnih malformacija primale kompletnu kemoterapiju tijekom cijele trudnoće. Prvi slučaj. Žena od 19 godina koja je spontano zanijela i krivo shvatila znakove trudnoće kao recidiv bolesti. Tijekom trudnoće je nastavila liječenjem, primivši do petog mjeseca smjesu hidroksiureje i -interferona i zatim do termina imatinib. Javila se s 38–39 tjedana trudnoće te je carskim rezom rodila savršeno zdravu malu djevojčicu težine 3510 grama. Krvna slika majke i djeteta je bila potpuno normalna. Drugi slučaj. Sličan slučaj mlade žene s limfoblastičnom akutnom leukemijom, liječenom vinkristinom, metotreksatom, purinetolom. Javila se u uznapredovalom porodu s 33–34 tjedana te je rodila djevojčicu tešku 1700 grama, bez malformacija i s normalnom krvnom slikom. Treći slučaj. Djevojka od 17 godina kojoj je s 29 tjedana trudnoće dijagnosticirana akutna mijeloična leukemija. Primila je indukcijsku kemoterapiju Ara-C-om, zbog značajne infiltracije koštane srži te bolešću uzrokovane diseminirane intravaskularne koagulopatije. S 32 tjedna počeli su trudovi te je carskim rezom rodila nedonošena dječačića težine 1750 grama s Apgar zbrojem 8. Dijete nije imalo malformacija ni klinički niti ultrazvučnim pregledom. Krvna slika je bila normalna. Do sada su studije pokazale da kronična mijeloična leukemija, liječena imatinibom, u 50% slučajeva rezultira rađanjem zdrava djeteta te da kemoterapija akutne leukemije tijekom drugog i trećeg tromjesečja trudnoće na zahtijeva prekid trudnoće, jer se može postići remisija bolesti i rađanje normalna djeteta

Translation of the Fugl-Meyer assessment into Romanian: Transcultural and semantic-linguistic adaptations and clinical validation

PurposeThe Fugl-Meyer Assessment (FMA) scale, which is widely used and highly recommended, is an appropriate tool for evaluating poststroke sensorimotor and other possible somatic deficits. It is also well-suited for capturing a dynamic rehabilitation process. The aim of this study was to first translate the entire sensorimotor FMA scale into Romanian using the transcultural and semantic-linguistic adaptations of its official afferent protocols and to then validate it using the preliminary clinical evaluation of inter- and intra-rater reliability and relevant concurrent validity.MethodsThrough three main steps, we completed a standardized procedure for translating FMA's official afferent evaluation protocols into Romanian and their transcultural and semantic-linguistic adaptation for both the upper and lower extremities. For relevant clinical validation, we evaluated 10 patients after a stroke two times: on days 1 and 2. All patients were evaluated simultaneously by two kinesi-physiotherapists (generically referred to as KFT1 and KFT2) over the course of 2 consecutive days, taking turns in the roles of an examiner and observer, and vice versa (inter-rater). Two scores were therefore obtained and compared for the same patient, i.e., being afferent to an inter-rater assay by comparing the assessment outcomes obtained by the two kinesi-physiotherapists, in between, and respectively, to the intra-rater assay: based on the evaluations of the same kinesi-physiotherapist, in two consecutive days, using a rank-based method (Svensson) for statistical analysis. We also compared our final Romanian version of FMA's official protocols for concurrent validity (Spearman's rank correlation statistical method) to both of the widely available assessment instruments: the Barthel Index (BI) and the modified Rankin scale (mRS).ResultsSvensson's method confirmed overall good inter- and intra-rater results for the main parts of the final Romanian version of FMA's evaluation protocols, regarding the percentage of agreement (≥80% on average) and for disagreement: relative position [RP; values outside the interval of (−0.1, 0.1) in only two measurements out of the 56 comparisons we did], relative concentration [RC; values outside the interval of (−0.1, 0.1) in only nine measurements out of the same 56 comparisons done], and relative rank variation [RV; all values within an interval of (0, 0.1) in only five measurements out of the 56 comparisons done]. High correlation values were obtained between the final Romanian version of FMA's evaluation protocols and the BI (ρ = 0.9167; p = 0.0002) for FMA–upper extremity (FMA-UE) total A-D (motor function) with ρ = 0.6319 and for FMA-lower extremity (FMA-LE) total E-F (motor function) with p = 0.0499, and close to the limit, with the mRS (ρ = −0.5937; p = 0.0704) for FMA-UE total A-D (motor function) and (ρ = −0.6615; p = 0.0372) for FMA-LE total E-F (motor function).ConclusionsThe final Romanian version of FMA's official evaluation protocols showed good preliminary reliability and validity, which could be thus recommended for use and expected to help improve the standardization of this assessment scale for patients after a stroke in Romania. Furthermore, this endeavor could be added to similar international translation and cross-cultural adaptations, thereby facilitating a more appropriate comparison of the evaluation and outcomes in the management of stroke worldwide

REGULATORY T CELLS AND THE MICROENVIRONMENT OF THE MALIGNANT B CELL OF CHRONIC LYMPHOCYTIC LEUKEMIA

In recent years understanding and modulating the tumor microenvironment (MT) has been the focus of a scientifically and clinically intense study. The role of T regulatory cells (Tregs) were investigated in terms of the suppression of tumor-specific immune responses and the establishment of an immunosuppressive tumor microenvironment (1). Regulatory T cells have a fundamental function in maintaining immune homeostasis in healthy individuals, and in cancer and in particular in haematological malignancies they seem to play a rather controversial role. Furthermore an increased frequency of Treg cells has been associated with tumor progression and has been correlated with an increased risk of death and reduced survival (2). The role of T cells in the pathogenesis of chronic lymphocytic leukemia has recently gained special attention due to the constant interaction between neoplastic B cells with the micromedium substrate and T cells. There is often a relatively large number of regulatory T cells in lymphoid tissues of CLL patients, that could affect the normal immune function (3)

Idiopathic thrombocytopenic purpura (ITP) – new era for an old disease

Immune thrombocytopenia is an autoimmune hematological disorder characterized by severely decreased platelet count of peripheral cause: platelet destruction via antiplatelet antibodies which may also affect marrow megakaryocytes. Patients may present in critical situations, with cutaneous and/or mucous bleeding and possibly life-threatening organ hemorrhages (cerebral, digestive, etc.) Therefore, rapid diagnosis and therapeutic intervention are mandatory