We present the images of peripheral blood smears of a patient diagnosed with plasma cell leukemia. Plasma cell leukemia is a rare and aggressive disease, characterized by more than 20% plasma cells in the peripheral blood or/and an absolute count of more than 2000/mm3.
Cirrhosis is the late stage of several hepatic disorders. The patients with cirrhosis show profound anomalies in their hemostatic system, every phase being affected. These changes result in an increased risk for both bleeding and thrombosis. The hemostatic anomalies include alteration of primary and secondary hemostasis and of fibrinolysis, namely: thrombocytopenia and platelet dysfunction, deficiency of synthesis or clearance of coagulation factors, reduced synthesis of natural anticoagulants, hyper- and hypofibrinolysis. The laboratory findings do not accurately assess the risk and are often misleading. Therefore, it is difficult to appreciate the risk of bleeding or thrombosis in cirrhotic patients. Bleeding is often variceal, but can also occur from peptic ulcers, Mallory-Weis syndrome or mucosal bleeding. The most frequent thrombotic complications are portal vein thrombosis, deep vein thrombosis and thromboembolism. The management of bleeding and thrombosis, and also the prophylaxis in the setting of invasive procedures are particularly challenging in cirrhotic patients. We present a review of hemostatic changes, their clinical outcome and the management in patients with cirrhosis.
We present the images of peripheral blood smears of a patient diagnosed with plasma cell leukemia. Plasma cell leukemia is a rare and aggressive disease, characterized by more than 20% plasma cells in the peripheral blood or/and an absolute count of more than 2000/mm3.
Primary immune thrombocytopenia (ITP) is characterized by isolated low platelet count and it is a diagnosis of exclusion, contrasting to secondary ITP. Therefore, a positive diagnosis is difficult and requires extensive investigation. Some of the underlying conditions that are associated with ITP are lymphoproliferative disorders and infections, especially viral ones. In the present study, the case of a patient diagnosed with diffuse large B‑cell lymphoma, who received chemotherapy and autologous hematopoietic stem cell transplantation is presented. After a complete remission of four years, the patient presented with sudden intense hemorrhagic syndrome and severely decreased platelet count. The most frequent causes of secondary ITP were excluded, including lymphoma relapse, and intravenous corticosteroids were started. However, shortly after hospital admission, the patient developed neuro‑psychiatric anomalies, fever and pancytopenia, and West‑Nile encephalitis was diagnosed. Although the initial development was favorable, he started to complain of progressive severe muscle weakness and eventually succumbed to infectious complications in the setting of prolonged hospitalization, corticotherapy, and immobilization.
ATL is a rare but a highly aggressive T-cell neoplasm associated with human T-cell leukemia virus-1 (HTLV-1) infection. Human T-cell lymphotropic virus type-1 (HTLV-1) is a oncogenic retrovirus responsible for the development of adult T-cell leukemia (ATL), but also for other non-malignant diseases, such as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). HTLV-1 has a higher prevalence in Japan, the Caribbean, South America, intertropical Africa, Romania, and northern Iran. ATL patients can have an extensive spectrum of neurological manifestations. Numerous factors can be implicated, such as central nervous system infiltrates, neurolymphomatosis, complications to medication or allogeneic stem cell transplantation, HAM/TSP, infections, metabolic disturbances. The neurological complications are not always easy to recognize and treat. Thus, this review underlines the necessity of a multidisciplinary approach in ATL patients with neurological symptomatology.
Background and Objectives: Hematological malignancies are usually systemic diseases of life-threatening impact, and frequently require prompt and energetic therapeutic intervention. Due to systemic involvement, the role of surgery is generally limited to diagnostic approaches, and it is very rarely employed as a therapeutic modality. Splenectomy represents an exception to this paradigm, being used both as a diagnostic and tumor debulking procedure, notably in splenic lymphomas. Materials and Methods: We investigated the role of splenectomy in a single center prospective study of splenectomy outcome in patients with splenic involvement in the course of lymphoproliferative disorders. In the present study, we included all patients treated in our department for lymphoid malignancies over a period of six years, who underwent splenectomy as a diagnostic or debulking procedure after referral and workup, or had been referred to our department after first being splenectomized and diagnosed with splenic lymphoma. Patient characteristics and treatment outcome were investigated. Results: We enrolled 54 patients, with 34 (63%) splenectomized patients: 12 splenectomies (22.2%) for diagnostic purposes and 22 (40.7%) for treatment. Special attention was given to the 28 (51.85%) patients diagnosed with splenic marginal zone lymphoma (SMZL), a subtype with a clear therapeutic indication for splenectomy. Average age of patients was 57.5 (±13.1) years with a higher prevalence of feminine gender (66.67%). Age above 60 years old (p = 0.0295), ECOG (Eastern Cooperative Oncology Group) > 2 (p = 0.0402) and B-signs (p nonsignificant (NS)) were most frequently found in SMZL patients. Anemia, and notably autoimmune anemia, was more frequent in SMZL versus other small-cell lymphomas and also in splenectomized patients, as was leukocytosis and lymphocytosis. Treatment of patients with lymphoproliferative disorders consisted of chemotherapy and/or splenectomy. Most SMZL patients received chemotherapy as first line treatment (61.5%) and had only partial response (57.7%). Second treatment line was splenectomy in 80% of patients who required treatment, followed by a 60% rate of complete response (CR). Splenectomy offered a higher complete response rate (twice as high than in non-splenectomized, regardless of histology type, p = NS), followed by a survival advantage (Overall Survival (OS)~64 versus 59 months, p = NS). Particularly, SMZL patients had a 4.8 times higher rate of CR than other non-Hodgkin lymphoma (NHL) patients (p = 0.04), a longer progression free survival (73 months vs. 31 months for other small-cell NHLs p = NS) and a 1.5fold lower death rate (p = NS). The procedure was rather safe, with a 38.5% frequency of adverse reactions, mostly minor and manageable. Conclusions: Our data suggest that splenectomy is an effective and safe therapeutic option in patients with lymphoid malignancies and splenic involvement, particularly splenic marginal zone lymphoma.
Abstract Objectives To assess the frequency of multiple thrombophilia-associated mutations and polymorphisms in a selected population of high-risk pregnancies. Methods Thrombophilia screening was performed for 1,500 pregnant women with prior pregnancy complications or thrombotic events. Nine thrombophilia-associated mutations or polymorphisms were screened: factor V Leiden, factor V H1299R, prothrombin G20210A, MTHFR C677T, MTHFR A1298C, factor XIII V34L, PAI-1 4G/5G polymorphisms, EPCR G4600A, EPCR C4678G. Results Out of the 1,500 patients, 1,291 fulfilled the criteria for data interpretation. All patients had low-risk thrombophilia-associated genetic variants. Only 1.24 % of cases presented high-risk abnormalities (homozygous factor V Leiden/prothrombin G20210A, or both mutations in heterozygous form). Heterozygous factor V Leiden occurred in 10.38 % of cases, while only 5.81 % carried heterozygous prothrombin G20210A mutation. The frequency of prothrombin G20210A mutation was higher (10.37 %) in the subgroup associating factor V Leiden, than in the subgroup lacking it (5.36 %). Low-risk genetic variants occurred with a higher frequency: 23.78 % factor V H1299R, 57.32 % MTHFR C677T, 55.54 % MTHFR A1298C, 44.07 % factor XIII V34L, 73.20 % PAI-1 4G/5G polymorphisms, 69.64 % EPCR G4600A, and 69.63 % EPCR C4678G. Conclusions All patients had at least one prothrombotic genetic mutation or variant. Our data highlight the need for thrombophilia screening, including low-risk genetic variants, in a high-risk population of pregnant women with a history of pregnancy complications or thrombotic events.
Metastatic cancers, particularly those involving bone marrow metastases, present significant challenges in clinical management. The presence of bone marrow metastases critically influences cancer treatment strategies, necessitating comprehensive patient assessments. Diagnostic confirmation involves imaging studies and bone marrow biopsy or aspiration, with histopathological and immunohistochemical exams. The prognosis for patients with bone marrow metastases is generally poor. Complications from myelosuppression and advanced disease stages further complicate the management. This article presents two cases of solid tumors with bone marrow metastases, highlighting the diverse presentations of such metastases. These cases emphasize the importance of bone marrow evaluation in patients with solid tumors who exhibit unexplained blood abnormalities, as this assessment is crucial for accurate diagnosis, informed treatment decisions, and improved prognosis.
Myelofibrosis is a chronic BCR-ABL1-negative myeloproliferative neoplasm characterized by bone marrow fibrosis, inefficient hematopoiesis and extramedullary hematopoiesis. Since their approval in the treatment of myelofibrosis, JAK inhibitors had a positive impact, being the most effective treatment for improving symptomatology and spleen reduction. However, one should weight the benefits and the risks of this treatment, which include hematological toxicity, opportunistic infections, reactivation of latent infections, and an increased risk for chronic lymphoproliferative disorders. We report two cases of myelofibrosis patients treated with a JAK2 inhibitor who developed non-Hodgkin lymphoma and tuberculosis, respectively.
Eosinophilia is a frequent laboratory abnormality, associated with many hematological malignancies, solid tumors or with autoimmune diseases. It is most often found in Hodgkin lymphoma and in T-cell lymphoma. In T-cell lymphoma or adult’s T-cell leukemia, it is highly correlated with the peripheral leukemic cell count, disease extension and with Ann-Arbor stage. Hypereosinophilic syndrome (HES) is a heterogeneous group of clonal disorders characterized by significant eosinophilia without an underlying cause, and with clinical features due to eosinophilic infiltration of lungs, heart or serous membranes. HES can precede lymphoma’s onset, being associated with a poor prognosis. The paraclinical investigations are necessary to determine the organ damage and various cytogenetic and molecular assays are employed to identify rearrangements of PDGFRA, PDGFRB, FGFR1 or PCM1-JAK2 gene fusion. The upcoming case report describes a patient with a non-Hodgkin T-cell lymphoma associating HES, treated with multiple chemotherapeutic regimens. After each cycle, he obtained an unstable response. It was considered a highly aggressive disease with a poor prognosis and a severe evolution.
