Natural history of clonal haematopoiesis seen in real-world haematology settings.

Recursive partitioning of healthy consortia led to the development of the Clonal Hematopoiesis Risk Score (CHRS) for clonal haematopoiesis (CH); however, in the practical setting, most cases of CH are diagnosed after patients present with cytopenias or related symptoms. To address this real-world population, we characterize the clinical trajectories of 94 patients with CH and distinguish CH harbouring canonical DNMT3A/TET2/ASXL1 mutations alone ('sole DTA') versus all other groups ('non-sole DTA'). TET2, rather than DNMT3A, was the most prevalent mutation in the real-world setting. Sole DTA patients did not progress to myeloid neoplasm (MN) in the absence of acquisition of other mutations. Contrastingly, 14 (20.1%) of 67 non-sole DTA patients progressed to MN. CHRS assessment showed a higher frequency of high-risk CH in non-sole DTA (vs. sole DTA) patients and in progressors (vs. non-progressors). RUNX1 mutation conferred the strongest risk for progression to MN (odds ratio [OR] 10.27, 95% CI 2.00-52.69, p = 0.0053). The mean variant allele frequency across all genes was higher in progressors than in non-progressors (36.9% ± 4.62% vs. 24.1% ± 1.67%, p = 0.0064). This analysis in the post-CHRS era underscores the natural history of CH, providing insight into patterns of progression to MN.

Perioperative Plasma Exchange and Intravenous Immunoglobulin Use for Refractory Heparin-Induced Thrombocytopenia in a Liver Transplant Recipient.

BACKGROUND Heparin-induced thrombocytopenia (HIT) is a serious adverse effect of heparin, which can lead to a prothrombotic state. Prompt cessation of heparin and initiation of non-heparin anticoagulation is the standard of care for HIT. Nevertheless, the treatment can pose challenges, particularly in refractory HIT, in patients with contraindications to anticoagulation, or those requiring urgent surgery. Additionally, in rare cases, conventional anticoagulation therapy is not effective, necessitating alternative treatments such as plasma exchange (PLEX) and intravenous immunoglobulin (IVIG). CASE REPORT Here, we report the case of a 57-year-old male patient who developed mild acute cellular rejection, refractory HIT, and disseminated intravascular coagulation after liver transplant surgery. Heparin was stopped and argatroban was initiated for thromboembolism treatment, but hepatic artery thrombosis occurred in the setting of refractory HIT and caused transplant failure. The patient underwent a second liver transplant 1 month after the first surgery. He had 2 sessions of PLEX and received 1 dose of IVIG before and 1 dose during the operation. Despite advanced treatment with PLEX and IVIG, the refractory HIT persisted. Hepatic artery thrombosis recurred within 2 weeks and the transplant failed again despite catheter-directed intra-arterial thrombolysis and argatroban therapy. CONCLUSIONS Recently perioperative PLEX and IVIG have been used a few times for the treatment of refractory HIT. This is the first reported case of a liver transplant recipient with refractory HIT who underwent this treatment strategy. Further investigation is required to determine the efficacy and safety of preoperative and intraoperative administration of PLEX and IVIG, especially in liver transplant recipients with HIT.

A Rare Case of Extramedullary Relapse of Multiple Myeloma Causing Bowel Obstruction.

Extramedullary myeloma, defined by presence of plasma cells outside the bone marrow, is a rare entity accounting for about 3-9% of all cases. It usually is aggressive with a median survival of <6 months. It is also associated with adverse prognostic factors including 17p deletions and high-risk gene profiles. While common extramedullary sites include bones, there have been several case reports of hematogenous extramedullary myeloma to the liver, lungs, pancreas, breast, skin, and soft tissues. Extramedullary myeloma to the mesentery is a rare entity with only a handful of cases reported. We present a case of 69-year-old man presenting with relapse of multiple myeloma to the mesentery, resulting in bowel obstruction to highlight the various presentations of myeloma.