EJHaem最新文献

{"title":"Primary plasmablastic plasma cell leukemia: A diagnostic conundrum","authors":"Radu Chiriac, Juliette Fontaine","doi":"10.1002/jha2.1085","DOIUrl":"10.1002/jha2.1085","url":null,"abstract":"<p>A man in his 60s presented with a fever (39.9°C), persistent fatigue, and abnormal weight loss. Laboratory investigations revealed hypercalcemia (3.7 mmol/L), elevated LDH (880 U/L), elevated serum creatinine (2.8 mg/dL), along with anemia (67 g/L), thrombocytopenia (50 × 10<sup>9</sup>/L), and 38% of circulating atypical-appearing lymphoid cells, varying in size and morphology, with inconspicuous nucleoli, cytoplasmic projections, and basophilic cytoplasm (Figure 1A). The absence of microscopic features characteristic of mature plasma cells hindered precise identification.</p><p>Bone marrow core biopsy showed massive infiltration by monomorphic, medium-sized cells with large, irregular nuclei and scant basophilic cytoplasm. The sample also exhibited a characteristic “starry-sky” appearance. Immunohistochemical staining (Figure 1B) revealed that the neoplastic cells were positive for CD138, MUM1, CCND1, BCL2, c-MYC, and kappa light chain, but negative for CD19, CD20, CD38, CD56, BCL6, ALK, and Human Herpesvirus-8 (HHV-8). The Ki-67 proliferation index was nearly 100%, with overexpression of p53 and c-MYC, and a confirmed <i>MYC</i> rearrangement (Figure 1B, inset). In situ hybridization for Epstein-Barr virus (EBV)-encoded RNA was negative. Additionally, fluorescence in situ hybridization analysis of enriched plasma cells demonstrated a del(17)(p13.1) involving <i>TP53</i> in 95% of cells (Figure 1C). HIV serology was negative. Serum protein electrophoresis showed no M spike but indicated hypogammaglobulinemia. The serum kappa/lambda free light chain ratio was 33.3. magnetic resonance imaging findings showed diffuse degenerative changes in the lumbar spine without vertebral collapse.</p><p>Given the phenotype (loss of pan-B-cell markers and expression of plasmacytic differentiation markers), bone lesions, absence of EBV and HHV-8 involvement, and myeloma-related secondary cytogenetic abnormalities (17p deletion and <i>MYC</i> translocation), the diagnosis was more consistent with plasmablastic myeloma (PBM).</p><p>An autologous stem cell transplant was planned, but after the third cycle of Dara-VRd induction, the patient tested positive for severe acute respiratory syndrome coronavirus 2. Two weeks later, he was transferred to the ICU for acute respiratory distress syndrome and, a month later, died from respiratory complications due to multidrug-resistant <i>Pseudomonas aeruginosa</i> ventilator-associated pneumonia.</p><p>The case demonstrates primary plasma cell leukemia with an uncommon plasmablastic morphology. The clinical presentation resembles that of acute leukemia and contrasts with classic plasma cell myeloma. The diverse morphologies of plasma cell neoplasms often mimic other hematopoietic and non-hematopoietic tumors, leading to their characterization as the “great imitator” [<span>1</span>].</p><p>Differential diagnostic considerations for PBM include lymphoid neoplasms with plasmablastic, immunoblastic, or plasmacytoid features, ","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756962/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143049004","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Oral squamous cell carcinoma in a patient affected by chronic graft versus host disease","authors":"Sem Decani, Giulia Ghidini, Niccolò Lombardi, Laura Moneghini, Elena Maria Varoni","doi":"10.1002/jha2.1069","DOIUrl":"10.1002/jha2.1069","url":null,"abstract":"<p>A 51-year-old Hispanic man with a history of chronic graft versus host disease (cGVHD) was referred, by his onco-haematologist, to our clinics because of a fast-growing lingual mass, in the absence of local trauma. The patient, a non-smoker, received, 4 years before, allogeneic hematopoietic stem cell transplantation (HSCT) from a human leukocyte antigen-id family donor for a stage IVB Epstein-Barr virus-related angioimmunoblastic T-cell lymphoma. After the HSCT, the patient developed cGVHD, with involvement of the liver, skin and oral mucosa. He was under current therapy with Sirolimus because of the worsening of hepatic values of stasis and necrosis related to the liver cGVHD. He was under follow-up and peritoneal dialysis for a drug-induced chronic renal failure.</p><p>Intraoral examination showed, at the left tongue apex, an exophytic, hard, red-whitish mass, surrounded by an erythroplasic lesion with a granular, irregular surface, extended to all the left side of the lingual dorsum (Figure 1). The lesion was painful during palpation. A keratotic white plaque was also detected on the lingual dorsum associated with severe tissue atrophy. Further keratotic white striae and erosions were observed at buccal and palatal mucosae, as signs of cGVHD.</p><p>Multiple incisional biopsies of the exophytic lingual mass and of the surrounding red area were performed. The histopathological analyses showed a micro-infiltrating oral squamous cell carcinoma (OSCC) in the context of epithelial dysplasia (Figure 2). After the oncological staging, the patient received surgical intervention and radiation therapy.</p><p>At a 2-year follow-up, the patient did not show any sign of recurrences.</p><p>Bone marrow transplanted patients are at high risk of developing solid malignancies, which can occur in up to 15% of cases and are responsible for 5%–10% of late deaths [<span>1, 2</span>]. The mechanisms involved in cancer onset may be the results of the combined presence of cGVHD (that can occur in 60%–80% of HSCT survivors and increase the risk of OSCC 35 times), radiation and chemotherapy regimens, chronic inflammation and long-term immunosuppression [<span>3-5</span>]. Oral cGVHD, in particular, is present in 80%–90% of patients affected by systemic cGVHD and it usually occurs as lichenoid lesions, as in this report. Oral cGVHD predisposes patients to OSCC even several years after HSCT, in the range from 1 to 22 years after the transplantation [<span>4, 6</span>]. Consistently, our patient developed oral cancer after 4 years. These patients require lifelong multidisciplinary oral cancer screening, and the role of onco-haematologists is pivotal to guarantee OSCC early diagnosis.</p><p>Sem Decani, Elena Maria Varoni and Niccolò Lombardi followed the patient during diagnosis, collected the clinical data, and reviewed the manuscript. Giulia Ghidini collected clinical data and wrote the report. Laura Moneghini performed the histopathological diagnosis. Written informed","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756986/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048983","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"CD58 expression does not impact response to inotuzumab ozogamicin in patients with B-cell acute lymphoblastic leukemia","authors":"Rafael Madero-Marroquin,&nbsp;Ryan W. Hunter,&nbsp;Caner Saygin,&nbsp;Hannah Johnston,&nbsp;Adam S. DuVall,&nbsp;Hamed Rahmani Youshanlouei,&nbsp;Clinton Osei,&nbsp;Syed Shah,&nbsp;Wendy Stock,&nbsp;Sandeep Gurbuxani,&nbsp;Anand A. Patel","doi":"10.1002/jha2.1076","DOIUrl":"10.1002/jha2.1076","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>CD58 loss has been described as a mechanism of resistance to blinatumomab and chimeric antigen receptor T-cell therapy, functioning as a modulator of response to T-cell activation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Using flow cytometry, we evaluated the impact of CD58 mean fluorescence intensity (MFI) on the probability of achieving measurable residual disease (MRD) negativity in patients with B-cell acute lymphoblastic leukemia treated with inotuzumab ozogamicin (InO).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The odds ratio of achieving MRD negativity was 1.03 for every 1000 unit increase in CD58 MFI.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Our results suggest that MRD negativity rates after InO are high, regardless of the intensity of CD58 expression.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756963/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048791","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Central nervous system posttransplant lymphoproliferative disorder following allogeneic hematopoietic stem cell transplantation successfully treated with combination therapy of acalabrutinib and immunochemotherapy: A case report and literature review","authors":"Peihao Zheng,&nbsp;Teng Xu,&nbsp;Xiaona Zuo,&nbsp;Xiaoyan Ke,&nbsp;Kai Hu","doi":"10.1002/jha2.1078","DOIUrl":"10.1002/jha2.1078","url":null,"abstract":"<p>Here, we report a case of Epstein-Barr virus-positive central nervous system-post-transplant lymphoproliferative disorder (CNS-PTLD) patient who failed to achieve complete metabolic remission (CMR) after successively trying a methotrexate-based regimen combined with orelabrutinib or whole-brain radiotherapy and encountered intracranial hemorrhage during orelabrutinib treatment. Ultimately, the patient achieved CMR after one cycle of acalabrutinib in combination with temozolomide, teniposide, liposomal doxorubicin, dexamethasone, and rituximab (TEDDi-R). Following another cycle of TEDDi-R treatment, he has been receiving acalabrutinib maintenance up to now and remained in CMR. The case may provide an effective treatment option for CNS-PTLD patients in clinical practice.</p>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756970/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048792","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"What is the origin of the normal ranges of blood cell counts? An evolutionary perspective","authors":"Manon Zala,&nbsp;Vincent Alcazer,&nbsp;Laetitia Largeaud,&nbsp;Pierre Sujobert","doi":"10.1002/jha2.1073","DOIUrl":"10.1002/jha2.1073","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>The normal values of the complete blood count are part of the foundational medical knowledge that is seldom questioned due to their well-established nature. These normal values are critical for optimal physiological function while minimizing the harmful consequences of an excessive number of blood cells. Thus, they represent an evolutionary trade-off likely shaped by natural selection if they significantly influence individual fitness and exhibit heritability.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>On the basis of the analysis of normal blood count values of 94 mammalian species, we discovered that certain parameters are strongly associated with diet, habitat, and lifespan.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Carnivorous mammals had higher hemoglobin levels than vegetarians, and aquatic mammals displayed red blood cell parameters probably selected to enhance for the diving capacities. Body weight influenced platelet counts and innate immune cells, with lighter animals having higher platelet counts and larger animals showing elevated monocytes and neutrophils.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>By treating the history of life as an experiment, we have discerned some evolutionary constraints likely contributing to the selection for optimal trade-offs in blood cell count.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756989/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048276","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The long-term risk of immune-related conditions in survivors of diffuse large B-cell lymphoma: A Danish nationwide registry study","authors":"Laura Schou Pedersen,&nbsp;Nadja Nørholm Klausen,&nbsp;Jonas Faartoft Jensen,&nbsp;Emilis Danielsen Bacevičius,&nbsp;Peter Brown,&nbsp;Judit Mészáros Jørgensen,&nbsp;Thomas Stauffer Larsen,&nbsp;Christian Bjørn Poulsen,&nbsp;Michael Roost Clausen,&nbsp;Robert Schou Pedersen,&nbsp;Anne Ortved Gang,&nbsp;Rasmus Westermann,&nbsp;Salome Kristensen,&nbsp;Lene Wohlfahrt Dreyer,&nbsp;Tarec Christoffer El-Galaly,&nbsp;Lasse Hjort Jakobsen","doi":"10.1002/jha2.1070","DOIUrl":"10.1002/jha2.1070","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>There is limited knowledge of the long-term effects on the immune system after treatment for diffuse large B-cell lymphoma (DLBCL).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This study included DLBCL patients from the Danish Lymphoma Registry who obtained complete remission (CR) after (R)-CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone)-like immunochemotherapy. Each R⁺ CHOP-like treated patient was matched to five comparators from the Danish background population and furthermore compared to R⁻ CHOP-like treated patients. Incidence rate ratios (IRRs) and risk differences (RDs) were calculated for a wide range of infections, autoimmune conditions, and immune deficiencies (AC-IDs) combined and by subtypes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>R⁺ CHOP-like treated patients had a higher risk of infections overall (IRR 1.5, 95% confidence interval [CI] 1.4–1.7: 10-year RD 5.0%, 95% CI 2.2%–7.8%) and for a majority of the subtypes than matched comparators. Likewise, they had a higher risk of AC-IDs overall (IRR 1.4, 95% CI 1.1–1.7; RD 0.8%, 95% CI 0.7%–2.2%) than matched comparators, however only of clinical relevance for three subtypes; autoimmune diseases of the endocrine system, sarcoidosis and immune deficiencies. The addition of rituximab to CHOP-like therapy did not alter the incidence rates (IR) of infections overall (IRR 1.1, 95% CI 0.9–1.3) or AC-IDs overall (IRR 0.8, 95% CI 0.5–1.3) compared to CHOP-like therapy alone, although the IR for respiratory infections was significantly elevated (IRR 1.5, 95% CI 1.1–2.1). However, an increased use of IVIG treatment was observed among R⁺ CHOP survivors.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>R-CHOP-like treated patients face an increased risk of infections and AC-IDs overall compared with the background population. The risk of infections and AC-IDs did not change overall after the addition of rituximab to CHOP, however, an increased risk of respiratory infections is notable. These findings could highlight the need for expanded vigilance and prophylaxis strategies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756995/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048972","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Single-center off-label benralizumab use for refractory hypereosinophilic syndrome demonstrates satisfactory safety and efficacy","authors":"Yvonne Veltman,&nbsp;Anna M. Aalbers,&nbsp;Maud A. W. Hermans,&nbsp;Pim G. N. J. Mutsaers","doi":"10.1002/jha2.1014","DOIUrl":"10.1002/jha2.1014","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Benralizumab is an interleukin 5-receptor-blocking drug registered for the treatment of eosinophilic asthma. It has proven efficient and safe in a small phase-II trial in hypereosinophilic syndrome and is currently being investigated in a larger, randomized phase-III trial. We report on real-world experience with benralizumab in 15 patients with severe Hypereosinophilic syndrome (HES) that were refractory to other treatments or on unacceptable steroid doses.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Fifteen patients with severe HES were treated with compassionate need benralizumab. The measured endpoints were a reduction in peripheral eosinophil count, a reduction of corticosteroid use, adverse events, and clinical response.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>All subgroups of HES were represented in this cohort and in the case of reactive HES, treatment of the primary cause did not lead to resolution of the eosinophilia. The median time of follow-up was 38 months. Twelve patients reached a normalized peripheral eosinophil count (&lt; 0.05 × 109/L), while the remaining three patients also had a significant reduction from baseline.</p>\u0000 \u0000 <p>Of the eight patients initially treated with steroids, five patients were off steroids completely, and three patients had reduced dosages. Eight patients experienced complete symptom resolution, and five partial resolution. No serious adverse events were observed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>In conclusion, benralizumab is safe and effective for the treatment of HES.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756971/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143049028","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Factors related to mean corpuscular volume in HFE p.C282Y homozygotes","authors":"James C. Barton,&nbsp;J. Clayborn Barton,&nbsp;Ronald T. Acton","doi":"10.1002/jha2.1063","DOIUrl":"10.1002/jha2.1063","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>The aim of this study was to define the relationships between factors other than transferrin saturation (TS) to mean corpuscular volume (MCV) and macrocytosis (MCV &gt; 100 fL) in <i>HFE</i> p.C282Y (rs1800562) homozygotes.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We studied white post-screening participants with p.C282Y homozygosity who did not have anemia, report cirrhosis or pregnancy, or use medications that increase MCV. We analyzed relations of MCV and macrocytosis with age, sex, diabetes reports, daily alcohol consumption, swollen or tender 2nd/3rd metacarpophalangeal (MCP) joints, TS, and serum ferritin (SF).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>There were 257 participants (110 men and 147 women). Median alcohol consumption, median TS, median SF, and macrocytosis prevalence were significantly greater in men. Relative risk of macrocytosis in men was 2.81. In men and women, there were significant positive Pearson's correlations of MCV versus age and Spearman's correlations of MCV versus alcohol consumption and TS. Mean MCV and macrocytosis prevalence were significantly greater in participants with than without swollen or tender 2nd/3rd MCP joints. Linear regression on MCV revealed positive associations: age (<i>p</i> &lt; 0.0001), alcohol consumption (<i>p</i> = 0.0007), and TS (<i>p</i> &lt; 0.0001). Logistic regression on macrocytosis revealed an odds ratio for age of 1.04 (95% confidence interval: 1.00, 1.07).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>MCV in <i>HFE</i> p.C282Y homozygotes is positively related to age, daily alcohol consumption, TS, and swollen or tender 2nd/3rd MCP joints.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756985/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048814","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Masked polycythemia vera in a patient 5 years after gastric bypass surgery: A diagnostic pitfall","authors":"Daniel Rivera,&nbsp;Carlos E. Bueso-Ramos,&nbsp;L Jeffrey Medeiros,&nbsp;Beenu Thakral","doi":"10.1002/jha2.1068","DOIUrl":"10.1002/jha2.1068","url":null,"abstract":"&lt;p&gt;A 66-year-old woman presented with a fever and symptoms of urinary tract infection. A complete blood count showed: white blood cells (WBC) 26.2 × 10&lt;sup&gt;9&lt;/sup&gt;/L(normal, 4–11 × 10&lt;sup&gt;9&lt;/sup&gt;/L), red blood cells (RBC) 7.2 × 10&lt;sup&gt;12&lt;/sup&gt;/L(normal 3.99–5.46 × 10&lt;sup&gt;12&lt;/sup&gt;/L), hemoglobin 12.4 g/dL(normal, 12.2–15.3 g/dL), hematocrit 45.3%(normal 36.4%–46.8%), mean corpuscular volume 63 fL(normal, 82–99 fL) and platelets 631 × 10&lt;sup&gt;9&lt;/sup&gt;/L(normal, 160–397 × 10&lt;sup&gt;9&lt;/sup&gt;/L). Serum iron was 15 µg/dL and ferritin was 10 ng/mL. A review of the peripheral blood smear (panel A) showed microcytic RBCs and pencil cells on the peripheral blood smear suggestive of iron deficiency. However, the high RBC count did not fit with iron deficiency, and thus bone marrow evaluation was performed. The biopsy specimen was hypercellular (∼90%) with panmyelosis and pleomorphic megakaryocytic hyperplasia without dysplasia or increased blasts (panels B–D). Absent storage iron was seen on iron stain performed on an aspirate smear. Further work-up showed that serum erythropoietin (EPO) was low and a detailed review of the medical history revealed the patient underwent gastric bypass surgery ∼5 years ago. Ultrasound abdomen detected mild splenomegaly (15.5 cm). Next-generation sequencing study showed &lt;i&gt;JAK2&lt;/i&gt; p.V617F(VAF:83%) mutation and conventional cytogenetics showed a normal diploid female karyotype. These results confirmed the diagnosis of “masked” polycythemia vera (PV) with concurrent iron deficiency likely due to gastric bypass (Figure 1).&lt;/p&gt;&lt;p&gt;According to the current World Health Organization classification and the International Consensus Classification, the diagnosis of PV requires three major criteria or two major with one minor criterion [&lt;span&gt;1, 2&lt;/span&gt;]. The three major criteria are 1) An elevated hemoglobin (≥16.5 g/dL in men and ≥16.0 g/dL in women) or elevated hematocrit (≥49% in men and ≥48% in women); 2) Bone marrow biopsy specimen with age-adjusted hypercellularity with panmyelosis and pleomorphic megakaryocytic proliferation, and 3) Presence of &lt;i&gt;JAK2&lt;/i&gt; p.V617F or &lt;i&gt;JAK2&lt;/i&gt; exon 12 mutation; the minor criterion is a subnormal EPO level [&lt;span&gt;1, 2&lt;/span&gt;]. Gastric bypass surgery compromises iron absorption by bypassing the portion of the small intestine where iron is absorbed, thus decreasing gastric acid production as a feedback loop. Furthermore, calcium and zinc may compete with iron for absorption and cause iron deficiency. Normal hemoglobin and hematocrit levels in the setting of chronic iron deficiency create a diagnostic pitfall when trying to meet diagnostic criteria for PV [&lt;span&gt;3, 4&lt;/span&gt;]. This scenario is a diagnostic pitfall and can delay the diagnosis of PV or even result in misdiagnosis as essential thrombocythemia or pre-fibrotic myelofibrosis. Awareness and knowledge of gastric bypass as an etiology of iron deficiency can help in the appropriate diagnosis, management, and risk stratification in PV patients. To","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756979/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048891","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association of the nutritional risk index recorded prior to allogeneic hematopoietic cell transplantation with the clinical prognosis in children","authors":"Hitomi Yonesu,&nbsp;Satoru Hamada,&nbsp;Hideki Sakiyama,&nbsp;Shinobu Kiyuna,&nbsp;Tokiko Oshiro,&nbsp;Nobuyuki Hyakuna,&nbsp;Koichi Nakanishi","doi":"10.1002/jha2.1054","DOIUrl":"10.1002/jha2.1054","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>The nutritional risk index (NRI), calculated using serum albumin levels and body weight ratio is a known prognostic factor in adult hematopoietic cell transplantation (HCT). However, its usefulness in pediatric HCT settings remains unclear.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In a retrospective study, we examined pre-transplant NRI impact on outcomes in 82 pediatric patients undergoing allogeneic HCT.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The 2-year non-relapse mortality (NRM) rate was 7.94% (95% confidence interval [CI], 3.05%–19.8%) and 30.8% (95% CI, 16.7%–52.2%) in the high and low NRI groups, respectively (<i>p</i> = 0.0037).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>We found that poor nutritional status prior to pediatric HCT led to a worse prognosis, including increased NRM.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756969/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143048789","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}