Lymphoma
Anju Sahdev, Sarah J. Vinnicombe in Husband & Reznek's Imaging in Oncology, 2020
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.
Gastrointestinal system
Aida Lai in Essential Concepts in Anatomy and Pathology for Undergraduate Revision, 2018
Causes of splenomegaly Congestive heart failureBacterial infection (TB)Viral infection (Epstein–Barr)CirrhosisPortal hypertensionPortal vein thrombosisThalassaemiaMegaloblastic anaemiaChronic myeloid leukaemia (CML)SLESarcoidosisAmyloidosisLymphoma
Short answer questions (SAQs)
Tristan Barrett, Nadeem Shaida, Ashley Shaw, Adrian K. Dixon in Radiology for Undergraduate Finals and Foundation Years, 2018
The spleen measures approximately 11–13 cm on average and is located beneath the ninth to twelfth ribs. Spleen size varies with age, sex and height, but most accept splenomegaly as being > 13 cm. The causes of splenomegaly are divided by size: massive (> 20 cm), moderate, or mild, or by the pathological mechanism. Small spleen may be due to SCD (auto-infarcts), infarction, atrophy, polysplenia syndromes. Causes of splenomegaly include: Congestive: CCF, portal hypertension / cirrhosis, CF.Infective: CMV, malaria, Epstein-Barr virus.Cancer: leukaemia, lymphoma, myelofibrosis, metastases.Storage disorders: Gaucher disease, amyloidosis, sarcoid.Connective tissue disorders: RhA (Felty’s syndrome), SLE.Haemolytic anaemias: hereditary spherocytosis.
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.
The Importance of Characterizing the Hemoglobin Instability of New Variants: The Case of Hb Dompierre [β29(B11)Gly→Arg, HBB: c.88G>C]
Published in Hemoglobin, 2020
Etienne Mondesert, Muriel Giansily Blaizot, Olivier Tournilhac, Anne François Serre Sapin, Bernard Aubin, Jean-Luc Pellequer, Patricia Aguilar Martinez
The instability of Hb Dompierre may explain the acute clinical manifestations observed in the present patient and the presence of some hemolytic biological features. Abdominal pain may be attributable to hemolytic crises, but is usually encountered in cases of massive intravascular hemolysis [10]. Despite the family history of abdominal pain on the maternal side, this does not seem to be associated with the Hb variant, which was not inherited from the mother. Other possible main causes of splenomegaly in this patient have been ruled out, leaving us with the hypothesis that it may have been caused by chronic hemolysis and triggered the fluctuating thrombocytopenia. Biological signs, such as the presence of spherocytes and the history of low haptoglobin, might indicate the presence of chronic hemolysis. Abnormal hemolytic parameters do not seem to be permanently present in this patient, as the measurements carried out at our laboratory, and again a few weeks after the clinical manifestations, were nearly normal. The presence of stigmata of inflammation (infection) might possibly explain the acute manifestations, as infections and some drugs can trigger acute hemolytic crises in patients who are carriers of an unstable Hb.