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Surgery of portal hypertension
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Caroline Lemoine, Riccardo A. Superina
Uncomplicated patients are followed as outpatients 1 month after surgery, then every 3 months for a year, and then yearly for 5 years. Resolution of splenomegaly is monitored through serial physical examination. A CBC, an ammonia level, and an abdominal Doppler US are performed at each appointment. The US evaluates both the patency of the shunt, and the velocities through the anastomoses. If there are no complications, a magnetic resonance imaging study is performed at the 1-year mark. This study provides an estimated blood flow through the venous conduit. Antiplatelet therapy is continued for 6 months after the surgery and then usually discontinued. Patients who suffer postoperative complications or have a known underlying hypercoagulable condition may require more frequent follow-up and cross-sectional imaging, and prolonged anticoagulation and/or antiplatelet therapy.
Lymphoma
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Sarah J Vinnicombe, Rodney J Hicks
Most patients with HL present with painless asymmetrical lymph node enlargement, accompanied by B symptoms (drenching night sweats, fever >38°C, and weight loss >10% of body weight in the 6 months prior to diagnosis) in about 40%. Pruritis and alcohol-induced pain can also occur. Clinically, the commonest site of nodal disease is the neck. Axillary nodal involvement occurs in 6%–20% of patients. Inguinal/femoral nodal disease is seen in 6%–15%, but exclusive infradiaphragmatic lymphadenopathy occurs in under 10% at diagnosis. Splenomegaly is found clinically in about 30% of patients. HL tends to spread in a contiguous fashion from one lymph node group to the next. Primary extranodal HL is very rare and should only be diagnosed after a thorough search for disease elsewhere.
The Lymphatic/Immune System and Its Disorders
Published in Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss, Understanding Medical Terms, 2020
Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss
Splenomegaly is enlargement of the spleen and is a symptom rather than a disease. The most common cause of splenomegaly is congestion. Many infectious processes, liver disease, and hematologic abnormalities can enlarge the spleen.
Pharmacotherapeutic advances for splenomegaly in myelofibrosis
Published in Expert Opinion on Pharmacotherapy, 2023
Douglas Tremblay, John Mascarenhas
Myelofibrosis (MF) is a chronic hematologic malignancy classified as a myeloproliferative neoplasm (MPNs), a group of interrelated disorders that share clinical and pathologic features [1]. MF can be primary (PMF) or secondary to an antecedent essential thrombocythemia (post-ET MF) or polycythemia vera (post-PV MF). Approximately 80% of MF patients have palpable splenomegaly at time of presentation [2] and 38% have a spleen that is palpable 10 cm below the left costal margin (LCM) [3]. Splenomegaly can cause symptoms including left upper quadrant abdominal pain and early satiety due to compression of the stomach. In addition, massive splenomegaly can lead to venous compression, manifesting as lower extremity edema and can also result in the development of portal hypertension. Splenic infarct can arise as a result of non-thrombotic ischemia which can be associated with significant abdominal pain and even hemodynamic instability [4]. Given the associated symptoms, improving spleen size is considered a major treatment goal by both physicians and patients, as well as regulatory agencies [5].
JAK2 V617f in chronic myeloid leukemia: driving force or passive bystander?
Published in Hematology, 2022
Francesco Tarantini, Cosimo Cumbo, Antonella Zagaria, Elisa Parciante, Luisa Anelli, Nicoletta Coccaro, Giuseppina Tota, Crescenzio Francesco Minervini, Immacolata Redavid, Antonella Russo Rossi, Maria Rosa Conserva, Giorgina Specchia, Pellegrino Musto, Francesco Albano
In 2007, a 49-year-old male was referred to our center for neutrophilic leukocytosis, unexplained weight loss and early satiety. His blood counts showed: white blood cells 77 × 109/L, hemoglobin 13.6 g/dL, platelets 380 × 109/L. A physical examination revealed splenomegaly. Peripheral blood (PB) smear showed the presence of myeloid precursor and ∼2% of blasts. Total RNA was extracted from bone marrow (BM) cells using the RNeasy Mini kit (Qiagen), and the presence of a b3a2 BCR-ABL1 fusion transcript was revealed by reverse-transcription PCR (RT–PCR) analysis. A BM aspiration and biopsy were consistent with a CML diagnosis. Conventional cytogenetic analysis of G-banded BM metaphase cells showed the following karyotype: 46,XY,t(9;22)(q34;q11.2)[20]. The Sokal risk score was intermediate (0,86). The patient was started on Imatinib Mesylate (IM) treatment at 400 mg/die. After 6 months, he reached a complete cytogenetic response. Molecular monitoring was initially performed by RT–PCR and then, starting from 2012, by real-time quantitative PCR (RQ-PCR) analysis using the BCR-ABL1Mbcr Kit (Ipsogen). The patient was regularly monitored in our outpatients clinic. In 2019, he expressed his will to stop IM and attempt treatment-free remission (TFR) [7]. At that time, he was in deep molecular response (MR4.5), with undetectable disease according to the international scale (IS) lasting4 years.
Splenic marginal zone B-cell lymphoma associated with ruptured breast implants: case report and review of the literature
Published in Case Reports in Plastic Surgery and Hand Surgery, 2021
Mark G. Evans, Melissa A. Mueller, Frederik Chen, Larry S. Nichter
Splenic marginal zone B-cell lymphoma is a rare malignancy, accounting for less than 2% of all lymphoma cases [12]. It was first described in 1992 and is now considered a separate entity in the World Health Organization (WHO) classification [13]. The three types of marginal zone B-cell lymphomas are splenic marginal zone lymphoma (SMZL), nodal marginal zone lymphoma (NMZL), and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). The diagnosis of SMZL is made based on lymphocyte morphology, immunophenotype, cytogenetic abnormalities, bone marrow histology, and spleen histology if available. When microscopic examination of the spleen is not possible, clinical splenomegaly and typical morphologic and immunophenotypic blood and bone marrow findings are sufficient to make a diagnosis.