62 research outputs found
Serum Immunoglobulins in non-Hodgkinās Lymphoma Patients
Serum proteins and immunoglobulin (Ig) findings in 119 non-Hodgkinās lymphoma (NHL) patients were analysed. Out of them 96 (81%) patients had B non-Hodgkin lymphoma (B-NHL), and 23 (19%) T-NHL. Indolent type of NHL was more frequent (77 patients, 65%), then aggressive type of NHL (42 patients, 35%). Most patients had normal serum protein concentration, the increased protein concentration was seen in 17% of patients while decreased concentration was noticed in 7% of patients. Hypoalbuminaemia was more frequent (43%) then hyperalbuminaemia (1%). In contrast to albumin, low levels of other protein fractions (alpha1-, alpha2-, and beta-globulin) were rather rare (0.6%, 4%, and 3% of patients, respectively) and high levels were frequent (23%, 37%, and 8%, respectively). Polyclonal hyperimmunoglobulinaemia was more frequent finding than hypoimmunoglobulinaemia. In 29% patients higher IgG level and in 25% patients higher IgA level were found. IgM hypoimmunoglobulinaemia (22%) was more frequent than IgG (11%) and IgA (8%) hypoimmunoglobulinaemia. M-spike in serum protein electrophoresis was found in 11 (7%) patients. The statistically significant association was not found between serum Ig concentration and lymphoma malignancy grade as well as between serum Ig concentration and immunologic origin of lymphoma. T-NHL patients have more often IgA concentration level above or under normal values than B-NHL patients (p<0.05)
WOUNDS IN HEMATOLOGY PATIENTS
Kod hematoloÅ”kog bolesnika rane mogu biti dio kliniÄke slike u trenutku postavljanja dijagnoze, posljedica infekcije, nuspojave terapije ili napredovanja tumorske bolesti s kožnim infiltratima. lijeÄenje rana kod hematoloÅ”kih bolesnika zahtijeva multidisciplinaran pristup hematologa, kirurga, dermatologa, mikrobiologa i ostalog medicinskog osoblja koje je ukljuÄeno u svakodnevnu brigu o bolesniku. Kako se radi o onkoloÅ”kim imunosuprimiranim bolesnicima, iznimno je važno pridržavati se mjera asepse te sprijeÄiti infekcije rana zbog kojih bi se zakomplicirao ionako dugotrajan oporavak i zalijeÄenje. Važno je na vrijeme prepoznati ranu s malignom infiltracijom jer je pravodobna kemoterapija u takvom sluÄaju kurativna mjera.Hematology patients can have wounds as part of the initial presentation of the disease, as a result of infection or therapy. Wound therapy is very important and requires multidisciplinary approach of the hematologist, surgeon, dermatologist, and all other medical staff involved in the patientās care. It is very important to provide aseptic care and prevent infections that could complicate the patientās recovery and cure. It is very important to recognize the wound with malignant infiltration because an appropriate chemotherapy can be curative
DIAGNOSTIC APPROACH AND TREATMENT OF IMMUNE THROMBOCYTOPENIA IN ADULTS
Cilj ovog preglednog rada jest prikazati najnovija saznanja povezana s definicijom imune trombocitopenije u odraslih (ITP), patoĀ¬fiziologijom bolesti, dijagnostiÄkim i terapijskim postupnikom. ITP je karakterizirana brojem trombocita manjim od 100x109/L te kroniÄnim tijekom u odsustvu neke druge bolesti koja bi mogla dovesti do smanjenja broja trombocita. Patogeneza ove bolesti temelji se na dva kljuÄna mehanizma: destrukciji kompleksa trombocit-protutijelo i inhibiciji sazrijevanja prekursora trombocita. Dijagnoza ITP-a temelji se na iskljuÄenju drugih moguÄih uzroka trombocitopenije. Odluka o poÄetku lijeÄenja donosi se individuĀ¬alno, prema broju trombocita (manji od 30x109/L) te riziÄnim faktorima krvarenja. Okosnica prve linije terapije su kortikosteroidi. U sluÄaju izostanka uspjeha lijeÄenja kortikosteroidima preporuÄa se primjena intravenskih imunoglobulina s brzim, ali Äesto i kratkotrajnim odgovorom. U drugoj liniji terapije su kirurÅ”ki i farmakoloÅ”ki pristupi. KirurÅ”ki pristup, splenektomija, karakteriziran je vrlo visokom stopom dugotrajnog odgovora. FarmakoloÅ”ki pristup danas znaÄi ili primjenu agonista trombopoetina (TPO) ili rituksimaba. Agonisti TPO pokazali su se kao lijekovi s visokom stopom odgovora, no potreba za kontinuiranim davanjem i cijena donekle ograniÄavaju njihovu upotrebu. Iako se rituksimab Äesto koristi u drugoj liniji lijeÄenja, za sada ne postoje randomizirana kliniÄka istraživanja koja bi poduprla njegovu primjenu. U bolesnika s refraktornim ITP-om lijeÄenje je potrebno samo u bolesniĀ¬ka s brojem trombocita manjim od 30x109/L te krvarenjem. Terapija je primjena agonista TPO, polikemoterapija, alemtuzumab te transplantacija perifernih matiÄnih stanica.The aim of this review is to provide the Croatian medical public with novel insights into the definition, pathogenesis, diagnostic algorithms and treatment approaches to immune thrombocytopenia (ITP) in adults. Recently, primary ITP has been uniformly deĀ¬fined as an autoimmune disorder characterized by an isolated platelet count lower than 100x109/L without preexisting disease or conditions, which could lead to thrombocytopenia. The recognition of primary and secondary ITP is important because they require different treatment strategies. In secondary ITP, therapeutic approach oriented towards the underlying disorder. Unlike childhood onset ITP, which is a self-limited condition with high rates of spontaneous remissions, adulthood onset ITP usually has chronic course. Previously, the pathogenesis of ITP was considered to be immune mediated destruction of platelets in liver and spleen, while recent findings have shown a novel pathophysiological pathway based on the inhibition of thrombopoiesis, leading to novel treatment approaches. The diagnosis of ITP is based on exclusion of the possible underlying causes of thrombocytopeĀ¬nia and consists of simple diagnostic procedures. The decision to treat ITP should be based individually: platelets count (lower than 30x109/L), various bleeding risk factors and patientās preference. The use of corticosteroids is the mainstay of first line therĀ¬apy. Two most commonly used corticosteroids are prednisone and dexamethasone. Prednisone is administered continuously, while dexamethasone is applied in cycles. Due to the lack of randomized clinical trials, it is not possible to recommend certain class of corticosteroid therapy. Another two agents used as first line therapy in case of corticosteroid refractoriness or the need of rapid platelet elevation, are intravenous immunoglobulins and anti-D immunoglobulin (anti-D is not approved in Europe). They are characterized by rapid onset of platelet recovery and low long-term remission rates.
Until recently, splenectomy, with adequate infectious and thromboprophylaxis, was the therapy of choice in patients who did not respond to corticosteroids due to high long-term remission rates and low relapse rates. This procedure can be offered to a younger patient without significant comorbidities after the first year of ITP duration. With advances in the understanding of ITP pathogenesis, a new class of drug has been established: thrombopoietin agonists (TPO). Eltrombopag and romiplostim, the TPO agonists currently approved for the management of ITP in patients who failed the first line therapy and are not suitable for splenectomy, are only two agents that have shown benefits in large clinical randomized trials. They are characterized by a high response rate and appropriate safety profile, but the need for continuous use, a high relapse rate after therapy withdrawal, and price limit their use in everyday practice. TPO agonists represent an appropriate treatment choice in patients who have relapse after splenectomy. Another agent, often used in everyday clinical practice, is rituximab with high response and relapse rates. Its use is based on small studies, and due to the lack of clinical randomized controlled trials, rituximab is not approved by the leadĀ¬ing medical agencies for this indication. As shown in this review article, our understanding and therapy for ITP has improved, but further research is needed to implement evidence-based therapy in clinical practice
Primary Gastrointestinal non-Hodgkin Lymphoma in Adults: Clinicopathologic and Survival Characteristics
Primary non-Hodgkin lymphomas of gastrointestinal tract (PGI-NHL) are the most common extranodal lymphomas with an increasing incidence. The incidence, clinicopathologic characteristics, treatment and survival were assessed in 39 successive, newly diagnosed PGI-NHL patients (23 male and 16 female) treated at Ā»MerkurĀ« University Hospital. The aim of the study was to precisely evaluate their characteristics and compare them with the results reported from other similar studies. The most common site of PGI-NHL was stomach (n=29, 74%), followed by small intestine (n=5, 13%), and colon and rectosigmoid (n=5, 13%). According to the Ann Arbor classification, 34 (87%) patients had stage IE and IIE, and five patients (12%) stage IIIE and IVE. According to World Health Organization (WHO) classification, 29 (87%) patients had diffuse large B-cell lymphoma (DLCBL), two had mantle cell lymphoma, and seven (18%) had marginal zone B-cell lymphoma-mucosa associated tissue (MALT). Twenty-six (66%) patients underwent surgical resection followed by chemotherapy, ten (26%) were treated with chemotherapy alone, and three (8%) were treated surgically. Complete remission was achieved in 28 (72%) and partial remission in seven (18%) patients. Four (10%) patients had progressive disease. In our patients, the major prognostic factor for outcome was the stage of disease. Patients with localized lymphoma (stage IE and IIE) had a significantly longer overall survival: 85% at five years and 65% at ten years. Patients with extended disease (stage IIIE and IVE) had overall survival less than 33%. The prognostic power of erythrocyte sedimentation rate (ESR), total protein, serum albumin, LDH concentration and activity was analyzed. Of these parameters, only LDH had a statistically significant effect on overall survival. In conclusion, our patient group was comparable to other literature reports on PGI-NHL patients according to clinicopathologic characteristics. Disease stage and LDH were the only parameters that had a statistically significant effect patient survival
T-Lymphoblastic Lymphoma with an Unusual t(8;14)(q24;q11) ā Case Report
Cytogenetic abnormalities seen at presentation of acute lymphoblastic leukemia or lymphoblastic lymphoma (ALL/ LBL) are associated with distinct clinical and hematologic disease entities. T-ALL/LBL are morphologically indistinguishable from those of B-ALL/LBL. An abnormal kariotype is found in 50ā70% of cases of T-ALL/LBL. We present a 35-year old male patient with T-ALL/LBL and t(8;14)(q24;q11.2). Our patient presented with B-symptoms, bulky mediastinal disease and CNS infiltration. Bone marrow was morphologically normal and cytogenetically without clonal aberrations. Cytological findings of the supraclavicular lymph node showed numerous CD3 positive (100%) and CD2 positive (88%) lymphoblasts, negative for CD34 and CD10. Flow cytometry of lymph node revealed T cell phenotype of im- mature cells: CD45+CD2+CD5+CD7+CD4+CD8+CD3cyt +CD3TdT+CD10-CD34-HLAD/DR-. Cytogenetic analysis of lymph node showed translocation t(1;4)(p32;p12), t(8;14)(q24;q11.2). Southern blot analysis of extracted DNA from the supraclavicular lymph node demonstrated clonal rearrangement of the T cell antigen receptor (TCR/J) gene (region Vb+Jb2). Based on these findings, diagnosis of T lymphoblastic non Hodgkin lymphoma was established. Cerebrospinal fluid analysis showed CNS infiltration with 49% lymphoblasts positive for CD4 and CD8. The disease progressed rapidly with poor response to therapy. T-ALL/LBL with an unusual t(8;14)(q24;q11.2) is a very rare hematologic disorder with rapid disease progression and poor response to conventional therapy because of frequent central nervous system involvement and early relapses
Myeloid Sarcoma Involving the Breast
Myeloid sarcoma is a tumor mass with extramedullary growth pattern, composed of myeloblasts or immature myeloid cells. The development of myeloid sarcoma may precede or concur with acute or chronic myeloid leukemia (AML or CML) or other myeloproliferative diseases or myelodysplastic syndromes (MDS). Isolated myeloid sarcoma of the breast is very rare. A case is presented of a 25-year-old, previously healthy woman that presented to our department for a palpable node, 5x2 cm in size, in the upper medial quadrant of her left breast. Fine needle aspiration (FNA) produced a sample consisting of medium sized blasts. Additional work-up revealed anemia, thrombocytopenia and leukocytosis, along with atypical blasts detected in peripheral blood and bone marrow smear. Based on the morphology, cytochemical characteristics and immature cell immunophenotype, it was considered a case of acute myeloid leukemia without maturation. In spite of intensive chemotherapy, the patient died within a year of diagnosis. In cases of isolated breast myeloid sarcoma, the diagnosis can be missed if the possibility of myeloid sarcoma is not remembered on differential diagnosis of a breast neoplasm
FOLLOW-UP OF PATIENTS WITH CLASSICAL HODGIN LYMPHOMA AFTER TREATMENT ā NOVEL EVIDENCE AND DILEMMAS. LITERATURE REVIEW
Cilj je ovoga preglednog rada prikazati suvremeno stajaliÅ”te struke, ali i prijepore u svezi s medicinskom skrbi za bolesnike s klasiÄnim Hodgkinovim limfomom u prvoj remisiji. VeÄina bolesnika s klasiÄnim Hodgkinovim limfomom izlijeÄena je prvom linijom terapije i u nastavku je važan medicinski pristup radi otkrivanja moguÄeg relapsa. Pregledom literature nismo naÅ”li jednoznaÄne smjernice za rutinsko praÄenje bolesnika slikovnim metodama, tj. kompjutoriziranom tomografijom ili pozitronskom emisijskom tomografijom. Prema dokazima u literaturi, otkriÄe asimptomatskog relapsa nije povezano s poboljÅ”anim ishodom lijeÄenja. Za sada se može smatrati da su standardne kliniÄke i radioloÅ”ke metode Ādostatne za praÄenje ovih bolesnika. Kasne toksiÄnosti uzrokovane mnogim lijekovima i zraÄenjem znatno pridonose morbiditetu bolesnika. To ukljuÄuje pojavu sekundarnih solidnih malignoma (ponajprije karcinoma pluÄa i dojke) i hematoloÅ”kih Āneoplazma koÅ”tane srži te pojavu benignih toksiÄnosti kao Å”to su poremeÄaji funkcije Å”titnjaÄe, fertiliteta i kardiovaskularnih bolesti. Usprkos obilju literature i smjernica za praÄenje ovih bolesnika nakon zavrÅ”etka lijeÄenja, za sada ne postoje rezultati prospektivnih istraživanja koji bi pružili temelj zasnovan na dokazima za nove smjernice i preporuke. Smatramo da otkrivanje kasne toksiÄnosti treba biti prilagoÄeno pojedinaÄnom bolesniku, sukladno specifiÄnom lijeÄenju i kasnijem utjecaju te toksiÄnosti na moguÄi morbiditet u ovih bolesnika.In this review we present current evidence for the follow-up of patients treated for classical Hodgkin lymphoma (HL). Nowadays introduction of novel therapies enabled successful treatment in most patients with classical HL in first remission with 5-year overall survival rate estimation of 80%. We have performed extensive literature search on the Āmethodological approach to detection of relapse. Evidence regarding imaging clinical methods in detecting relapse on Āserial computed tomography and/or positron emission tomography scans is scarce. These imaging modalities are associated with considerable economic cost, unnecessary exposure to radiation and patientsā stress. Furthermore, the detection of Āasymptomatic relapse does not seem to be associated with improved outcome in this patient group. Available data on this subject indicate that standard imaging methods, such as ultrasound, and judicious clinical examination in detecting of Ārelapse should be the basis of HL patient follow-up. Late toxicity due to various modalities of treatment represents serious morbidity in HL. They vary from secondary solid cancers and hematologic neoplasms, associated with poor outcome, to benign disorders (fertility issues, thyroid dysfunction, cardiovascular and lung disorders). Current data on the incidence, prevalence and etiological factors do not yet provide evidence on appropriate screening methods. Most recommendations in various guidelines are associated with low level of evidence (grade IV). We, therefore, propose individually-tailored screening methods for each patient based on the modality of treatment received
ARE WE ENTERING CHEMO-FREE ERA IN CHRONIC LYMPHOCYTIC LEUKEMIA? THE ROLE OF IBRUTINIB AND VENETOCLAX AND LESSONS LEARNT FROM IDELALISIB
Glavni cilj ovog preglednog rada je predstaviti novu klasu lijekova u kroniÄnoj limfocitnoj leukemiji (KLL), najÄeÅ”Äoj leukemiji odrasle dobi, inhibitore staniÄnog signaliziranja B receptora (BCR). KLL se klasiÄno lijeÄila imunokemoterapijom, no pojedini bolesnici (poodmakla životna dob, nepovoljni bioloÅ”ki faktori) imali su loÅ”u prognozu. S boljim razumijevanjem patogeneze unutarstaniÄnih putova pojavila se moguÄnost selektivne inhibicije i ciljane terapije u KLL. Prvi lijek u svojoj klasi ibrutinib, inhibitor bruton kinaze, pokazao se superiornim u fazama III kliniÄkih pokusa te je eliminirao negativne prognostiÄke faktore u lijeÄenju KLL, pogotovo del (17p), s adekvatnim profi lom toksiÄnosti Å”to su prepoznale regulatorne agencije. Drugi BCR inhibitori idelalisib i venetoklaks su iznimno aktivni u relapsnom okruženju, no idelalisib se pokazao neprihvatljivo toksiÄnim u prvoj liniji lijeÄenja Å”to nam može poslužiti kao lekcija u dizajnu kliniÄkih pokusa s ovim lijekovima. Usprkos uÄinkovitosti, potreban je dodatni napredak u ovom podruÄju koji se nalazi u kombinacijama s imunoterapijom ili imunokemoterapijom, te moguÄoj meÄusobnoj kombinaciji kako bismo dodatno poboljÅ”ali ishode. No, najveÄa zapreka BCR inhibitorima da uÄu u Å”iroku praksu je njihova cijena i utjecaj na zdravstveni sustav Å”to nažalost ograniÄuje naÅ”u moguÄnost da lijeÄimo bolesnike s KLL u eri bez kemoterapije.The main aim of this review is to present a novel class of agents, the inhibitors of B cell receptor (BCR) signaling pathway, used in the treatment of chronic lymphocytic leukemia (CLL) as the most common leukemia in the Western world. Traditionally, CLL was treated with immunochemotherapy, but certain subpupulations (elderly, biological prognostic factors) had poor outcome. With advances in our understanding the pathogenesis of intracellular pathways, the possibility of selective inhibition and targeted therapy in CLL has arisen. The fi rst agent in the class of BCR inhibitors, ibrutinib, a Bruton kinase inhibitor, has been shown superior in phase III clinical trials eliminating negative prognostic factors such as del(17p), with adequate toxicity profi le, which was recognized by the respective regulatory agencies. Other BCR inhibitors idelalisib and venetoclax are extremely active in relapsed setting, but unfortunately, idelalisib combinations in fi rst line clinical trials resulted in unacceptable toxicity, which is a cautionary tale on designing trials. Despite their effi cacy, we are only at the beginning to improve them by combination with monoclonal antibodies, immunochemotherapy, or between each other to improve outcomes of CLL treatment even further. However, the main obstacle to chemo-free era in CLL is their price resulting in limited access to these agents and inequity in the modern treatment of CLL
PraÄenje bolesnika s klasiÄnim Hodgkinovim limfomom nakon lijeÄenja ā suvremena saznanja i nedoumice. Pregled literature [Follow-up of patients with classical Hodgin lymphoma after treatment - novel evidence and dilemmas. Literature review]
In this review we present current evidence for the follow-up of patients treated for classical Hodgkin lymphoma (HL). Nowadays introduction of novel therapies enabled successful treatment in most patients with classical HL in first remission with 5-year overall survival rate estimation of 80%. We have performed extensive literature search on the methodological approach to detection of relapse. Evidence regarding imaging clinical methods in detecting relapse on serial computed tomography and/or positron emission tomography scans is scarce. These imaging modalities are associated with considerable economic cost, unnecessary exposure to radiation and patients' stress. Furthermore, the detection of asymptomatic relapse does not seem to be associated with improved outcome in this patient group. Available data on this subject indicate that standard imaging methods, such as ultrasound, and judicious clinical examination in detecting of relapse should be the basis of HL patient follow-up. Late toxicity due to various modalities of treatment represents serious morbidity in HL. They vary from secondary solid cancers and hematologic neoplasms, associated with poor outcome, to benign disorders (fertility issues, thyroid dysfunction, cardiovascular and lung disorders). Current data on the incidence, prevalence and etiological factors do not yet provide evidence on appropriate screening methods. Most recommendations in various guidelines are associated with low level of evidence (grade IV). We, therefore, propose individually-tailored screening methods for each patient based on the modality of treatment received
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