Blastic plasmacytoid dendritic cell neoplasm (BPDCN), recently classified among the acute myeloid leukemia and related precursor neoplasms, is a rare hematological malignancy with highly aggressive clinical course. We report a case of a 55 year-old female patient who presented spontaneous rupture of the spleen. The histopathological diagnosis without immunohistochemistry was splenic marginal zone non-Hodgkin lymphoma (NHL). The patient received 4 courses of polychemotherapy. Due to the unfavorable evolution the spleen was histopathologically reexamined and immunohistochemistry was performed in another laboratory. The diagnosis of NHL was excluded. Undifferentiated malignant cell proliferation, possible myeloid was suspected. Due to the discrepancy between diagnoses, a third immunohistological examination was performed in a third laboratory. NHL was excluded and myeloid, NK-cell or plasmacytoid dendritic cell leukemia was suspected. The patient was admitted to our clinic after 8 months from the initial diagnosis. Immunophenotyping by flowcytometry from the bone marrow showed 23% blasts positive for CD4, CD56, CD123, HLA-DR, CD38, CD11b, CD2, and negative for lineage specific markers of B-, T-lymphoid, NK- or myelomonocytic cells. The final diagnosis was BPDCN in leukemic phase. Due to the diversity of the clinical presentation, morphology and immunophenotype of BPDCN the diagnosis of this rare malignancy remains challenging. Immunophenotyping by flowcytometry is superior to immunohistochemistry because of the availability of a larger panel of antibodies and the possibility to identify the intensity of antigen expression. The atypical forms of BPDCN should be recognized early in order to manage properly the patients with this aggressive disease
Introduction: Autologous haemopoietic stem cell transplantation (SCT) is an important treatment modality for patients with acute myeloid leukemia with low and intermediate risk disease. It has served advantages over allogenic transplantation, because it does not need a matched donor, there is no graft versus host disease, there are less complications and a faster immune reconstitution than in the allo-setting. The disadvantage is the lack of the graft versus leukaemia effect.
Materials and methods: In the Bone Marrow Transplantation Unit Tîrgu Mureș 14 patients with acute myeloid leukemia received an autologous SCT. Mobilization of the stem cells was performed using chemotherapy and granulocytic colony stimulating factor. The conditioning regimen for SCT consists in monotherapy with busulfan (Bu) 16 mg/kg, BuCy: busulfan in combination with Cyclophosphamide (CY) 120 mg/ kg or BuMel: Busulfan in association with Melphalan (Mel) 140 mg/m2.
Results: The median patient age was 36 years (range 20-55), 9 (64%) were males and 5 (36) were females and the median time interval from diagnosis to autologous SCT was 9 months (range 3-25). All the patients were transplanted successfully, all of them achieved a sustained neutrophil count (> 0.5 G/L), median time 11 days (9-15) and platelet count (> 20 G/L) median time 14 days (10-19) after transplantation.
Conclusions: We conclude that autologous stem cell transplantation is an effective treatment in acute myeloid leukemia with the possibility of long survival, particularly in patients with standard risk disease
Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder, which can involve the hematopoietic stem cell or early progenitor cells, without the loss of their capacity to differentiate. Typically, CML has three clinical phases: a chronic phase, an accelerated phase, and an aggressive transformation in blast crisis, analogous to acute leukemia. The following article presents the case of a 49-year-old patient diagnosed with Philadelphia-negative CML in blastic transformation, where after multiple conventional acute leukemia induction chemotherapy regimens an unrelated allogeneic hematopoietic stem cell transplant was performed.
Introduction: Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder; the molecular hallmark of the disease is the BCR-ABL gene rearrangement, which usually occurs as the result of a reciprocal translocation between chromosomes 9 and 22. Tyrosine kinase inhibitors (TKI) were the first drugs that targeted the constitutively active BCR-ABL kinase and it have become the standard frontline therapy for CML. Monitoring the treatment of CML patients with detection of bcr-abl transcript levels with real time qualitative polymerase chain reaction (RQ-PCR) is essential in evaluating the therapeutic response.
Material and method: At the Clinical Hematology and BMT Unit Tîrgu Mureș, between 2008-2011, we performed the molecular monitoring of bcr-abl transcript levels with RQ-PCR in 16 patients diagnosed with CML.
Results: We have 11 patients on imatinib treatment who achieved major molecular response. One patient lost the complete molecular response after 5 years of treatment. Two patients in blast crisis underwent allogeneic hematopoietic stem cell transplantation from identical sibling donors. The first patient is in complete molecular remission after 4 years of the transplant with mild chronic GVHD. The other patient had an early relapse with treatment refractory disease and died from evolution of the disease. Three patients with advanced phases of the disease present increasing transcript levels. We performed the dose escalation, and for two of them the switch to the second generation of TKI.
Conclusions: Regular molecular monitoring of individual patients with CML is clearly desirable. It allows for a reassessment of the therapeutic strategy in cases of rising levels of BCR-ABL as an early indication of loss of response.
Introduction: Anemia is a common complication of malignant lymphomas, which could be a direct consequence of the disease or secondary to the myelosupressive chemotherapy. The aim of this study was to assess the effect of erythropoietin to treat anemia. The main objectives were to demonstrate increases in hemoglobin levels and the existence of an association between symptom relief and treatment.
Material and method: In the Clinical Hematology and BMT Unit Tîrgu Mureș we performed an analytical, observational study to assess the role of erythropoietin treatment in malignant lymphoma related anemia. This linear, retrospective study included 127 patients diagnosed and treated with malignant lymphoma between January 1st, 2007 and December 30, 2011. The 127 patients were divided into two groups: a group of patients (n = 88) who were treated with erythropoietin and the other group (n = 39) who did not receive this treatment. Patients included in the study received treatment with epoetin beta 40,000 IU/week. We followed the hemoglobin level and the symptomatology at baseline and after 4 weeks.
Results: Patients who received treatment with erythropoietin had a 7.12 times higher possibility of being asymptomatic than patients who did not receive this treatment. The hemoglobin concentration of patients with erythropoietin treatment increased significantly (p <0.0001) compared to the patients who did not receive this treatment.
Conclusion: Effective treatment of anemia is an important aim in the management of patients with malignant lymphomas, because it increases their hemoglobin concentration, decreases the need of transfusion and maintains an acceptable quality of life.
Background: There are several histologic variants and clinical subtypes of diffuse large B cell lymphoma, which includes the primary mediastinal large B cell lymphoma (PMBL). In the last 10 years the incidence of diffuse large lymphomas grew significantly.
Case report: We present the case and evolution of an aggressive life-threatening mediastinal B cell lymphoma with respiratory insufficiency, diagnosed in the 27th week of pregnancy. After 4 courses of R-CHOP the clinical status has somewhat improved, but the dyspnea, the facial and neck oedema and the trouble of speech persisted. After the patient was admitted to our hospital, she received DHAP regimen followed by mobilization with G-CSF. Before transplantation we administered another 3 courses of DHAP chemotherapy with spectacular results. We performed autologous hematopoietic stem cell transplantation preceded by BEAM chemotherapy. At present, 5 years post-transplant the patient is well, with no metabolically active disease on the PET-CT performed 3 months ago.
Conclusion: We can conclude that even in very complicated DLBCL cases, with a very good, efficient medical-team work we can salvage lives, in our case both of the mother and the child’s. Even in partially chemo-refractory cases like in the presented one, salvage chemotherapy followed by autologous transplantation can lead to a successful treatment.
Introduction: Plasmacytoid dendritic cell leukemia is a rare subtype of acute leukemia, which has recently been established as a distinct pathologic entity that typically follows a highly aggressive clinical course in adults. The aim of this report is to present a case of plasmacytoid dendritic cell leukemia due to its rarity and difficulty to recognize and diagnose it.
Case report: We present a case of a 67 year-old man who presented multiple subcutaneous lesions on his face, neck, chest and upper extremities with reddish-brown, brown colour. In the bone marrow aspirate 83% of the blast cells were found. Immunophenotypically the blasts were positive for CD4, CD56, CD123 (high intensity), CD36, CD22, CD10 (10.42%), CD33, HLA-DR, CD7 (9.24%), CD38 (34.8%) and negative for CD13, CD64, CD14, CD16, CD15, CD11b, CD11c, CD3, CD5, CD2, CD8, CD19, CD20, CD34. The skin biopsy showed lymphohistiocytoid infiltration in the dermis. The patient was diagnosed with acute plasmacytoid dendritic cell leukemia and received polychemotherapy with rapid response of skin lesions and blastic infiltration of the bone marrow. After 3 courses of polychemotherapy the cutaneous lesions reappeared and multiplied. The blast infiltration in the bone marrow increased to 70%. A more aggressive polychemotherapy regimen was administered, but the patient presented serious complications (febrile neutropenia) and died in septic shock 8 months after the initiation of treatment.
Conclusions: Immunophenotyping of blasts cells is indispensable in the diagnosis of plasmacytoid dendritic cell leukemia. The CD4+, CD56+, lin-, CD123 ++high, CD11c-, CD36+, HLA-DR+, CD34-, CD45+ low profile is highly suggestive for pDCL. The outcome of plasmacytoid dendritic cell leukemia is poor. Despite the high rate of initial response to treatment, early relapses occur and the patients die of disease progression.
Background: The elucidation of the genetic background of the myeloproliferative neoplasms completely changed the management of these disorders: the presence of the Philadelphia chromosome and/or the BCR-ABL oncogene is pathognomonic for chronic myeloid leukemia and identification of JAK2 gene mutations are useful in polycytemia vera (PV), essential thrombocytemia (ET) and myelofibrosis (PMF). The aim of this study was to investigate the role of molecular biology tests in the management of myeloproliferative neoplasms.
Materials and methods: We tested the blood samples of 117 patients between April 2008 and February 2013 at the Molecular Biology of UMF Târgu Mureș using RQ-PCR (for M-BCR-ABL oncogene) and/or allele-specific PCR (for JAK2V617F mutation).
Results: Thirty-two patients presented the M-BCR-ABL oncogene, 16 of them were regularly tested as a follow-up of the administered therapy: the majority of chronic phase patients presented decreasing or stable values, while in case of accelerated phase and blast phase the M-BCR-ABL values increased or remained at the same level. Twenty patients were identified with the JAK2V617F mutation: 8 patients with PV, 4 with ET, 3 with PMF, 4 with unclassifiable chronic myeloproliferative disease and 1 patient with chronic myelomonocytic leukemia. There was no case of concomitant occurance of both molecular markers.
Conclusions: Molecular biology testing plays an important role in the management of myeloproliferative neoplasms: identification of the molecular markers confirms the final diagnosis, excluding secondary causes of abnormal blood count parameters. Regular monitoring of MBCR- ABL expression level is useful in the follow-up of therapeutic efficiency.