There are no clear criteria for selecting elderly patients with hematological malignancies eligible for allogeneic hematopoietic stem cell transplantation. This study aimed to evaluate inflammatory and nutritional status biomarkers as prognostic indicators of allogeneic transplantation in elderly patients. We compared the prognostic effects of four representative pretransplant biomarkers: C-reactive protein-to-albumin ratio (CAR), Glasgow prognostic score (GPS), prognostic nutritional index (PNI), and albumin-to-globulin ratio (AGR). In total, 143 patients aged ≥ 60 years who underwent their first allogeneic transplantation for hematological malignancies were enrolled between 2010 and 2020 in our single-center cohort. The median patient age was 65 (range, 60-72) years. Pretransplant high CAR, high GPS, and low PNI scores were associated with poor overall survival (OS), whereas AGR was not associated with OS. CAR stratified the OS most significantly among the four biomarkers (p < 0.001). Multivariate analyses revealed that only high CAR was an independent prognostic factor associated with OS (hazard ratio [HR], 1.98; p = 0.031) and that hematopoietic cell transplant-specific comorbidity index (HCT-CI) score ≥ 3 was also associated with OS (HR, 2.04; p = 0.012). High CAR was correlated with poor performance status, male sex, and high disease risk index scores, but not with high HCT-CI scores. When the patients were stratified into three groups according to a composite risk assessment using CAR and HCT-CI, the 3-year OS significantly decreased with increasing scores (82.8%, 50.3%, and 27.0%, respectively; p < 0.0001). CAR is the most useful prognostic indicator among the inflammatory and nutritional status biomarkers for allogeneic transplantation in elderly patients. Inflammatory and nutritional status in the elderly may be important prognostic factors for allogeneic transplantation, independent of HCT-CI scores.

Rationale: Thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome are nonmalignant but life-threatening systemic inflammatory disorders. However, many patients are refractory to treatment, resulting in significant morbidity and mortality. Additionally, established treatment options are unavailable. Therefore, we present 2 cases of adults with the iMCD-TAFRO syndrome refractory to initial treatment but responded to Janus kinase (JAK) inhibitors with ruxolitinib. The report reveals that these rare adult cases of the refractory and treatment-resistant iMCD-TAFRO syndrome can be treated using JAK inhibitors. Patient concerns: Case 1 is a 36-year-old previously healthy male patient who presented with fever and general fatigue for 2 weeks. Case 2 is a 42-year-old previously healthy female patient who presented with fever and general fatigue. Diagnosis: The diagnosis met the 2015 criteria for TAFRO syndrome, as determined by All Japan TAFRO Syndrome Research Group in the Research Program for Intractable Disease by the Ministry of Health, Labor and Welfare (MHLW) Japan. Interventions: Treatment with tocilizumab and several immunosuppressants were ineffective. So, we performed ruxolitinib. Outcomes: Each patient received ruxolitinib, the general condition improved, and CRP levels decreased. Lessons: These cases showed that ruxolitinib was effective for treatment-resistant/ refractory TAFRO syndrome. Further prospective studies are needed on using ruxolitinib with a small number of cases.

TAFRO syndrome, first reported in 2010, is a systemic inflammatory disease with a rapid onset and potentially fatal course if not treated promptly and appropriately. The name is derived from the initial letters describing the characteristic symptoms of thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It is sometimes considered a special subtype of idiopathic multicentric Castleman disease (iMCD) because lymph node biopsies often reveal the pathology findings seen in iMCD. However, its clinical manifestations and prognoses are not well documented. Since the clinical manifestations and prognoses of TAFRO syndrome differ significantly from those of iMCD, it is recognized as an independent disease concept and considered to partially overlap with the pathology of MCD. The pathogenesis of TAFRO syndrome remains largely unknown. Due to the lack of appropriate treatment, it often presents with multiple organ dysfunction and fatality. In this review, we summarized new findings on the pathogenesis of TAFRO syndrome and discussed current effective therapies and future treatment strategies.

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal diffuse large B-cell lymphoma characterized by proliferation of B cells within small vessels. Herein, we report a case of a 77-year-old man who presented with IVLBCL and massive tumor formation on the aortic wall who was previously diagnosed with sarcoidosis and focal segmental glomerulosclerosis (FSGS). To our knowledge, this is the first reported case of an IVLBCL with aortic tumor formation. A 77-year-old ambulatory man with sarcoidosis and FSGS had neurological symptoms for nine months. The patient presented to the emergency department with sudden left leg pain, and was diagnosed with acute femoral artery occlusion. Emergency thrombectomy was performed subsequently. Pathological evaluation of the thrombi revealed that its surface was filled with large atypical B cells. Bone marrow biopsy showed infiltration of large atypical B cells within the small vessels. IVLBCL was suspected and further examination was planned, but the patient died due to sudden respiratory and cardiac arrest on hospital day twelve. Autopsy revealed intravascular tumors adherent to the aortic arch, left ventricle, and the abdominal aorta. All enlarged lymph nodes and the ventricular septum of the heart showed hyalinized lesions with granular formation consistent with sarcoidosis. The patient was diagnosed with IVLBCL with aortic tumor formation complicated with sarcoidosis and FSGS. IVLBCL may present with tumor formation on the aortic wall. Although the cause of its affinity to the aortic wall is yet unknown, autopsy findings imply that arteriosclerosis may have contributed to the tumor formation. The literature suggests that T-cell abnormalities could possibly be the common etiology of intravascular lymphoma, sarcoidosis, and FSGS.

A 50-year-old man was diagnosed with multiple myeloma complicating AL amyloidosis. Splenic rupture was complicated during autologous stem cell transplantation (auto-SCT). Granulocyte colony-stimulating factor (G-CSF) was not administered. A pathological examination of the spleen revealed that CD34-positive cells were concentrated in the ruptured part of the splenic capsule. Hematopoietic cells were engrafted in the small gap between the capsule and amyloid protein deposition area of the spleen, which might have caused the splenic rupture in the absence of G-CSF administration. Special attention is thus required for amyloidosis patients undergoing auto-SCT, even when G-CSF is not administered.