Péter Rajnics

Rare primary extranodal lymphomas: Diffuse large B-cell lymphomas of the genital tract

Primary non-Hodgkin’s lymphoma (NHL) of the genital tract is a rare entity. Etiology and pathogenesis of these NHLs are unknown, although there might be a possible association between chronic inflammation and lymphomas. The most common histological subtype is the diffuse large B-cell lymphoma. We report two cases of uterine lymphoma and one case of prostate lymphoma in this paper. The symptoms and the differential diagnosis are also discussed. Because of the low incidence, there is no widely accepted consensus on its treatment. We demonstrate that the rituximab and CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone; R-CHOP) chemoimmunotherapy is a good and tolerable treatment option in all cases. The two young patients are disease-free nowadays; the older patient with poor prognostic histological-type lymphoma relapsed in a short time and died after second relapse. A multicenter analysis is necessary to evaluate the long-term results of chemoimmunotherapy in these rare extranodal lymphoma entities.

Outcome of pregnancy in chronic myeloid leukaemia patients treated with tyrosine kinase inhibitors: Short report from a single centre

BACKGROUND The aim of this work was to report on the outcome of pregnancy in patients with chronic myeloid leukaemia who were on tyrosine kinase inhibitor treatment. PATIENTS AND METHODS We report the result of 22 pregnancies in 14 patients (9 female and 5 male) who conceived or their partner conceived while being on tyrosine kinase inhibitors for their CML. RESULTS All pregnancies except one were uneventful. 25 newborns were born and except in one case where small atrial septal defect was diagnosed, all infants were healthy and showed normal development after birth. CONCLUSION This small series does indicate that parents can most likely expect an uneventful outcome to a pregnancy despite exposure of either partner to TKIs. There is no consensus or guideline regarding the best practice in case of pregnancy. More reports of similar nature would certainly be beneficial to practitioners and patients alike. As such it is still recommended to practice effective contraception during the period of TKI treatment.

Az alacsony átlagos vörösvértest-hemoglobin alkalmas a vashiány szűrésére

Absztrakt Bevezetes: A vashiany szeles nepreteget erintő problema, szűrővizsgalata fontos egeszsegugyi kerdes. Celkitűzes: A szerzők arra a kerdesre kerestek valaszt, hogy a laboratoriumi automatak altal meghatarozott verkepparameterek kozul az alacsony MCH alkalmas-e a vashiany szűresere. Modszer: A Somogy Megyei Kaposi Mor Oktato Korhaz kozponti laboratoriumaban a 2013. januar 1. es 2015. junius 30. kozotti 30 honapos időszakban 247 705 verkepvizsgalat es 10 840 vasanyagcsere-vizsgalat tortent. A verkepvizsgalat alacsony atlagos vorosvertest-hemoglobinerteket hasonlitottak ossze az egyidejűleg elvegzett vashianyt igazolo parameterekkel. Eredmenyek: 830 betegben talaltak alacsony (28 pg alatti) atlagos vorosvertest-hemoglobinerteket es szimultan elvegzett vasanyagcsere laboratoriumi parametereket. Kozuluk 679 beteg eseteben (82%) az alacsony atlagos vorosvertest-hemoglobinertek vashiannyal jart egyutt. 126 hemodializalt (15%), 6 thalassaemias (minor), 8 myelofibrosisos, 5 rheumatoid arthritises beteg ese...

The Hematologic Toxicity of Methotrexate in Patients with Autoimmune Disorders

The incidence of auto-immune diseases is increasing nowa- days. Despite of the development of diagnosis and management of diseases, they remained chronic diseases. The patient’s lifespan expansion requires long-term treatment with harmful agents, such as Methotrexate or other immuno-suppressive drugs. The Methotrexate toxicities are based on the duration and cumulative dosing of drug, and the combination with other drugs. Myelosuppression and consequent pancytopenia is the most frequent hematologic toxicity, which occur mostly later during low dose methotrexate administration. We demonstrate three cases of low dose Methotrexate toxicity in older patients with rheumatoid arthritis and psoriasis. All patients were treated with low dose Methotrexate along more than one year continuously. Two old patients with RA and another with psoriasis developed pancytopenia causing severe neutropenia, cutaneous bleeding, and bruising and septic condition. They required intravenous antibiotic therapy, corticosteroids and limited transfusion dependence as a result of low dose Methotrexate. We have assessed the possible causes of Methotrexate toxicities and found that all patients used non-steroid anti-inflammatory drugs because of pain and protonpump inhibitor to avoid development of peptic ulcer. Two patients recovered, another died in septic condition. We would like to drawn attention of hematologists, dermatologists and rheumatologists to the harmful effect of low dose methotrexate in this patient population and emphasize the role of rigorous and consequent hematologic testing to avoid these severe late complications.

Low Mean Cell Haemoglobin is a Valuable Parameter of Thrombotic Risk Stratification in Patients with Polycythemia Vera

Objectives: Thrombosis is a leading cause of morbidity and mortality in patients with Philadelphia negative chronic myeloproliferative neoplasms (MPNs). There are many thrombosis risk stratifications used in this patient group taking into consideration the age, thrombosis history and cardiovascular factors (hypertension, hypercholesterinaemia, hyper-trigliceridaemia, thrombocytosis, smoking and diabetes mellitus). In this work we evaluated the possible role of iron deficiency in thrombotic events (TE) of the polycythaemia vera (PV) patients. Methods: We considered the low mean cell haemoglobin (MCH <28 pg) value as a parameter to assess the iron deficiency in the multicentre database (15 Hungarian haematology centres) of our HUMYPRON GROUP (Hungarian MPN Working Group). The MCH values, recorded at the time of diagnosis of 296 patients with polycythemia vera, were retrospectively analysed.Results: The low MCH, at the diagnosis, was found to be a risk factor for thrombotic events occurring after diagnosis (OR: 1.966). It was also shown as an additive and independent parameter in the Tefferi high-risk patient groups, and combining it with Tefferi risk stratification an extremely high thrombotic risk group could be determined (Nagelkerke R square: 0.084). We have supposed that low MCH in PV reveals a disease form featured with a high proliferation activity. Our hypothesis was confirmed with a sub-study (n=52) showing that the high JAK2V617F allele burden was significantly correlated with the low MCH (p=0.005) and the high white blood cell count (WBC) (p<0.001).Conclusions: Iron deficiency, existing at the time of diagnosis of PV, was proven to be a risk factor for imminent thrombotic events. The low MCH was found to be a strong additive factor when it was combined with the known thrombotic risk stratification systems. The low MCH showed significant correlation with the high JAK2V617F allele burden.

PET/MR in Relapsed Multiple Myeloma

Multiple myeloma is a hematologic malignancy characterized by a clonal proliferation of plasma cells. PET/MR is a new emerging hybrid imaging modality, its potential role in numerous different malignant diseases is under extensive evaluation. Our report describes PET/MR findings of a relapsed multiple myeloma case.

Imatinib therapy in chronic myeloid leukemia

UNLABELLED Chronic myeloid leukemia is a malignant clonal alteration of the pluripotent hemopoietic stem cell. The genetic hallmarks of the disease are the t(9,22) (Philadelphia chromosome), registered by conventional cytogenetics in more than 90% of all chronic myeloid leukemia cases and the active tyrosine kinase protein encoded by bcr-abl fusion gene. The constitutively active tyrosine kinase is currently accepted to be the cause of chronic myeloid leukemia. The introduction of imatinib has considerably changed the treatment of chronic myeloid leukemia. Prior studies demonstrated high rates of cytogenetic responses in all phases of the disease. METHODS The authors evaluated the cytogenetic and molecular responses of 21 chronic phase chronic myeloid leukemia patients who were consecutively admitted to their center. 13 of them were primarily treated with imatinib, and the other 7 were heavily pretreated with interferon alpha, cytarabine, all-trans-retinoic acid. Hydroxyurea pretreatment was routinely introduced in all patients until complete hematologic remission. Peripheral blood sample in every 3 months were collected for quantitative real-time polymerase chain reaction, and bone marrow aspirate in every 6 months for conventional cytogenetics. RESULTS Hematologic remission could have been achieved with hydroxyurea pretreatment in each patient. Complete cytogenetic remission at the 6th month and major molecular response at the 12th month were observed in each patient. CONCLUSIONS Imatinib treatment caused complete cytogenetic response and major molecular response in each chronic phase chronic myeloid leukaemia patient in our group. Hydroxyurea might have some effect on the rapid and deep cytogenetic and molecular responses, observed in the primary imatinib-treated group.A kronikus myeloid leukaemia a pluripotens haemopoeticus őssejt malignus klonalis betegsege. A korkep genetikai hattere a t(9;22) (Philadelphia kromoszoma), amely konvencionalis citogenetikaval a kronikus myeloid leukaemias esetek tobb mint 90%-aban kimutathato, illetve a bcr-abl fuzios gen, amely tirozin-kinaz-aktivitasu fuzios feherje szinteziset kodolja. Mai ismereteink alapjan ez a folyamatosan aktiv tirozin-kinaz a kronikus myeloid leukaemia oka. Az imatinib alkalmazasa jelentősen megvaltoztatta a kronikus myeloid leukaemia kezeleset. Az eddigi eredmenyek a betegseg minden fazisaban magas aranyu citogenetikai valaszt igazoltak. Modszerek: A szerzők az altaluk kezelt 21, kronikus fazisu kronikus myeloid leukaemias beteg citogenetikai es molekularis valaszait mutatjak be. Kozuluk 13 eseteben az imatinib első vonalbeli kezeles volt, a 7 masik beteg eseteben tobb szerrel (interferon-alfa, citarabin, all-trans-retinoic acid) tortent előkezelest kovetően alkalmaztak. Minden kronikus myeloid leukaemias bete...

Imatinib treatment of our chronic myeloid leukemia patients

UNLABELLED Chronic myeloid leukemia is a malignant clonal alteration of the pluripotent hemopoietic stem cell. The genetic hallmarks of the disease are the t(9,22) (Philadelphia chromosome), registered by conventional cytogenetics in more than 90% of all chronic myeloid leukemia cases and the active tyrosine kinase protein encoded by bcr-abl fusion gene. The constitutively active tyrosine kinase is currently accepted to be the cause of chronic myeloid leukemia. The introduction of imatinib has considerably changed the treatment of chronic myeloid leukemia. Prior studies demonstrated high rates of cytogenetic responses in all phases of the disease. METHODS The authors evaluated the cytogenetic and molecular responses of 21 chronic phase chronic myeloid leukemia patients who were consecutively admitted to their center. 13 of them were primarily treated with imatinib, and the other 7 were heavily pretreated with interferon alpha, cytarabine, all-trans-retinoic acid. Hydroxyurea pretreatment was routinely introduced in all patients until complete hematologic remission. Peripheral blood sample in every 3 months were collected for quantitative real-time polymerase chain reaction, and bone marrow aspirate in every 6 months for conventional cytogenetics. RESULTS Hematologic remission could have been achieved with hydroxyurea pretreatment in each patient. Complete cytogenetic remission at the 6th month and major molecular response at the 12th month were observed in each patient. CONCLUSIONS Imatinib treatment caused complete cytogenetic response and major molecular response in each chronic phase chronic myeloid leukaemia patient in our group. Hydroxyurea might have some effect on the rapid and deep cytogenetic and molecular responses, observed in the primary imatinib-treated group.A kronikus myeloid leukaemia a pluripotens haemopoeticus őssejt malignus klonalis betegsege. A korkep genetikai hattere a t(9;22) (Philadelphia kromoszoma), amely konvencionalis citogenetikaval a kronikus myeloid leukaemias esetek tobb mint 90%-aban kimutathato, illetve a bcr-abl fuzios gen, amely tirozin-kinaz-aktivitasu fuzios feherje szinteziset kodolja. Mai ismereteink alapjan ez a folyamatosan aktiv tirozin-kinaz a kronikus myeloid leukaemia oka. Az imatinib alkalmazasa jelentősen megvaltoztatta a kronikus myeloid leukaemia kezeleset. Az eddigi eredmenyek a betegseg minden fazisaban magas aranyu citogenetikai valaszt igazoltak. Modszerek: A szerzők az altaluk kezelt 21, kronikus fazisu kronikus myeloid leukaemias beteg citogenetikai es molekularis valaszait mutatjak be. Kozuluk 13 eseteben az imatinib első vonalbeli kezeles volt, a 7 masik beteg eseteben tobb szerrel (interferon-alfa, citarabin, all-trans-retinoic acid) tortent előkezelest kovetően alkalmaztak. Minden kronikus myeloid leukaemias bete...

[Long-term survival after nasal NK/T cell lymphoma].

The nasal NK/T cell lymphoma is a rare, extranodal non-Hodgkin lymphoma in western civilizations, which has poor prognosis. The Epstein-Barr virus can be detected in tumor cells in nearly all cases. There are no definite treatment guidelines in our days. There is no significant difference in survival between radiotherapy and chemotherapy according to Asian studies. In this case study we show our diagnostic procedures, our treatment options and we present the summary of this illness based on the data found in the literature.

Nazális NK/T-sejtes lymphoma hosszú túlélése@@@Long-term survival of the nasal NK/T cell lymphoma

The nasal NK/T cell lymphoma is a rare, extranodal non-Hodgkin lymphoma in western civilizations, which has poor prognosis. The Epstein-Barr virus can be detected in tumor cells in nearly all cases. There are no definite treatment guidelines in our days. There is no significant difference in survival between radiotherapy and chemotherapy according to Asian studies. In this case study we show our diagnostic procedures, our treatment options and we present the summary of this illness based on the data found in the literature.