Solid organ transplant-associated graft-versus-host disease (SOT-GVHD) is a rare but often fatal complication following liver transplantation. A 67-year-old man who underwent deceased donor liver transplantation subsequently developed progressive high-volume diarrhea and severe cytopenias with minimal initial cutaneous involvement. Colonic biopsy demonstrated apoptotic colitis with minimal lamina propria inflammation, suggestive of lower gastrointestinal (GI) GVHD. Donor-recipient chimerism analysis revealed significant donor-derived chimerism in peripheral blood and colonic tissue, confirming the diagnosis of SOT-GVHD. Maintenance immunosuppression was stopped to restore recipient immune function and eliminate donor-derived lymphoid cells; however, there was no clinical improvement. He was subsequently treated with high-dose systemic steroids, resulting in transient improvement. Despite this, he developed worsening diarrhea and pancytopenia, consistent with steroid-refractory GVHD, and passed away. This case emphasizes the need for a high index of suspicion for SOT-GVHD in liver transplant recipients presenting with gastrointestinal symptoms and cytopenias, even in the absence of significant early cutaneous manifestations. Histopathology and donor chimerism analysis of the involved tissue can aid in early diagnosis. Initial therapeutic approaches vary significantly, both withdrawing immunosuppression to elicit a host-versus-graft response and augmenting immunosuppression with systemic steroids have been described. Regardless, SOT-GVHD is associated with a high rate of mortality. Novel approaches for early diagnosis, prevention, and treatment are needed.

Hypersplenism in adults with homozygous sickle cell disease (HbSS) is rare, as most patients undergo autosplenectomy during early childhood. However, phenotypic variants such as high fetal hemoglobin, coexisting hemoglobinopathies, and different globin gene haplotypes can lead to splenic preservation and altered disease progression. These patients often exhibit milder symptoms, delayed complications, and increased morbidity. We report a rare case of a 43-year-old female with HbSS and elevated fetal hemoglobin who presented with hypersplenism, characterized by massive splenomegaly, pancytopenia, and compensatory bone marrow proliferation. She underwent an open splenectomy with an uneventful postoperative course. This case highlights the importance of recognizing atypical presentations in adult patients with sickle cell disease, in whom splenomegaly may persist. Clinicians should maintain a high index of suspicion to facilitate timely diagnosis and appropriate management, which can significantly improve patient outcomes.

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ dysfunction, with high untreated mortality that can be effectively reduced with timely plasma exchange (PLEX) and immunosuppressive therapy. However, several conditions can produce a clinically similar pseudo-TMA picture, among which severe vitamin B12 deficiency is an underrecognized and potentially treatable mimic that occurs due to ineffective intramedullary erythropoiesis, megaloblastic fragmentation of structurally abnormal erythrocytes, and pancytopenia in the absence of true endothelial injury or ADAMTS13 deficiency. We present a 74-year-old female patient who presented with altered mental status, bradycardia, and severe hemolytic anemia, in whom PLEX was initiated for suspected TTP but failed to improve platelet counts despite four sessions, ultimately prompting a workup that showed B12 deficiency due to pernicious anemia, confirmed by an elevated methylmalonic acid (MMA) and low serum B12, with complete hematologic recovery following cyanocobalamin repletion.

A 71-year-old woman was found to have pancytopenia during a routine health check-up and was referred to a local hospital. Bone marrow examination did not yield a definitive diagnosis, and the cytopenia gradually progressed over the following two years. She was subsequently referred to our institution for further evaluation, including assessment of eligibility for hematopoietic stem cell transplantation. [18]F-Fluorodeoxyglucose positron emission tomography ([18]F-FDG PET/CT) revealed massive splenomegaly without abnormal FDG uptake. Given the concurrent infection suspected to be fungal pneumonia, a splenectomy was performed for diagnostic and therapeutic purposes. Histopathological examination of the resected spleen, together with T-cell receptor gene rearrangement analysis, led to a diagnosis of hepatosplenic T-cell lymphoma (HSTCL). Following splenectomy, hematologic recovery was achieved rapidly, and CHOP chemotherapy was administered. The patient has remained in remission for approximately one year without evidence of relapse. Although HSTCL generally has a poor prognosis, this case is notable in that splenectomy both established the diagnosis and led to marked improvement in pancytopenia.

Pancytopenia is a significant haematological finding with a broad differential, ranging from benign conditions to life-threatening malignancies. Copper deficiency is a recognised but uncommon cause of non-clonal cytopenias. We report a case of copper deficiency in a woman in her 80s who presented with urolithiasis and was found to have acute pancytopenia. A thorough evaluation identified undetectable copper levels despite the absence of classic risk factors such as gastrointestinal surgery, malabsorptive disorders or zinc supplementation. Our patient's copper deficiency was most likely multifactorial arising from insufficient dietary intake and chronic mild gastrointestinal dysfunction. Emerging evidence suggests that hypocupremia may be more common than previously appreciated, and clinicians should consider copper deficiency in the differential diagnosis of pancytopenia.

Methotrexate, a commonly used disease-modifying anti-rheumatoid drug, rarely causes bone marrow suppression and pancytopenia. Consequently, we made a retrospective analysis of all patients who were admitted with pancytopenia following low-dose methotrexate intake between 2019 and 2025. Factors that were associated with the development of toxicity, clinical features and outcomes were evaluated. Among those who developed early toxicity, the majority had inadvertently taken daily instead of weekly doses. Renal failure, elderly age and hypoalbuminaemia contributed to delayed toxicity. All were given Folinic acid rescue therapy and 20% needed transfusion support. Two succumbed due to severe neutropenia-related sepsis. Simple patient education on once a week use, dose adjustment and frequent monitoring in the elderly and those with renal failure may help to reduce toxicity.

This all-case post-marketing surveillance study in Japan evaluated the safety and effectiveness of romiplostim, a thrombopoietin receptor agonist, in patients with aplastic anemia (AA), a rare disease characterized by pancytopenia. All patients with refractory AA who initiated romiplostim from June 2019 to September 2021 were registered. Romiplostim was administered subcutaneously once weekly for 52 weeks. Observation lasted 52 weeks or until 4 weeks after discontinuation. Occurrences of bone marrow fibrosis and hematopoietic tumors were monitored for up to 2 years after romiplostim initiation. Data from 470 patients at 220 institutions were analyzed. The mean age ± standard deviation was 66.27 ± 16.89 years, and 43.40% were male. Romiplostim was discontinued in 219/470 patients (46.60%). Adverse events (AEs) occurred in 57.02% of patients, and adverse drug reactions (ADRs) occurred in 18.09%. AEs of special interest classified as ADRs included thromboembolism (1.49%), hematopoietic tumor (1.28%), and hemorrhage (0.46%). During follow-up, hematopoietic tumors as ADRs were observed in 2/310 patients (0.65%). Hematological response was achieved in 257/327 patients (78.59%) at 27 weeks and 205/244 patients (84.02%) at 52 weeks. Platelet transfusion independence or reduced requirement was achieved in 164/213 patients (77.00%) at 27 weeks and 133/160 patients (83.13%) at 52 weeks, while red blood cell transfusion independence or reduced requirement was achieved in 194/247 patients (78.54%) and 162/186 patients (87.10%), respectively. Long-term romiplostim treatment was safe and effective in patients with refractory AA in a real-world setting. Registration: University Hospital Medical Information Network (UMIN000056465), 16 December, 2024.

Intracranial vertebral artery dissecting aneurysm is a rare type of intracranial aneurysm characterized by outward protrusion of the vessel wall due to damage to the intima and elastic membrane. Aplastic anemia is a relatively rare and severe syndrome involving bone marrow failure leading to pancytopenia. No previously reported cases exist of intracranial vertebral artery dissecting aneurysm complicated by aplastic anemia. Due to thrombocytopenia and coagulation dysfunction, managing concomitant vascular lesions during neurointerventional therapy poses significant challenges. We report the case of a 40-year-old man who with a history of aplastic anemia. Computed tomography angiography revealed a dissecting aneurysm in the right vertebral artery segment IV, with a distal branch to the posterior inferior cerebellar artery. The patient demonstrated favorable outcomes following individualized neurointerventional surgery with no complications. This case underscores the importance of developing personalized neurointerventional strategies for patients with intracranial aneurysms complicated by hematologic disorders. Priority should be given to controlling bleeding risks while maintaining posterior circulation patency, providing crucial guidance for clinicians managing such conditions.

Hepatic duct perforation (HDP) is a rare and life-threatening condition in adults, typically caused by direct injury or increases in intraductal pressure. The risk of HDP following cardiac surgery is poorly documented, although predisposing factors, such as bilirubin overload and ischemia from cardiopulmonary bypass, share common pathophysiological mechanisms with postoperative cholecystitis. We report the case of an 80-year-old woman who underwent mitral valve replacement for infectious endocarditis. Preoperatively, she was asymptomatic with mild common hepatic duct dilatation but no stones or ascites. However, her condition was also complicated by anemia and sepsis-induced disseminated intravascular coagulation. The cardiac surgery involved 153 minutes of extracorporeal circulation and required massive transfusion. On postoperative day (POD) four, she developed respiratory failure requiring re-intubation. A subsequent computed tomography and abdominal paracentesis confirmed HDP. Given her hemodynamic instability and prohibitive surgical risk, percutaneous drainage was performed. Although the acute biliary peritonitis initially improved, she developed persistent low-grade inflammation. On POD 61, she suffered from pancytopenia and reactivation of both varicella-zoster virus and cytomegalovirus, ultimately leading to multiple organ failure and death on POD 84. HDP can be a critical postoperative complication of cardiac surgery, potentially triggered by bypass-induced hypoperfusion and systemic stress. Despite successful percutaneous drainage, the persistent inflammatory state may induce secondary immunodeficiency and fatal opportunistic viral reactivation. This case highlights the inherent limitations of conservative management and the difficulty of deciding on definitive surgical intervention in critically ill postoperative patients.

Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome, is a life-threatening hyperinflammatory syndrome. Primary HLH is more common in children, while secondary HLH, triggered by malignancy, infection, or autoimmunity, predominates in adults. We report the case of a 56-year-old man referred to our institution with a 10-month history of fever (>38.3°C) meeting diagnostic criteria for fever of unknown origin (FUO). Clinical evaluation revealed splenomegaly and pancytopenia. Given the high clinical suspicion of HLH, an H-score of 219 points was calculated, and management was initiated. As part of the diagnostic workup, Ga-67 citrate scintigraphy and multimodal imaging were performed, revealing intense splenic uptake and structural abnormalities. These findings guided a targeted splenectomy, which confirmed classic splenic Hodgkin lymphoma (HL) (nodular sclerosis subtype). This case illustrates a systematic clinical approach to HLH, enabling the identification of the underlying etiology and facilitating timely intervention. The association between HLH and HL is rare and carries a high mortality rate; however, prompt etiological treatment significantly improves survival. We present, to the best of our knowledge, the first-ever reported case of HLH associated with splenic HL.