Real-World Outcomes of Ruxolitinib Treatment for Polycythemia Vera

IntroductionRuxolitinib is approved for the treatment of polycythemia vera (PV) with hydroxyurea resistance or intolerance. Approval was based on the phase III RESPONSE trial, which demonstrated efficacy in a highly selected patient population.Materials and MethodsTo characterize the tolerability and outcomes of ruxolitinib outside of a clinical trial, we performed a multi-center retrospective analysis of patients with PV treated with ruxolitinib at 11 participating sites across the United States. Outcomes of interest included change in phlebotomy requirements after starting ruxolitinib and spleen response, as these were included in the primary composite outcome in the RESPONSE trial.ResultsOne hundred twenty-six patients met eligibility criteria, and the median duration of follow-up was 22.4 months (range, 0-63.0 months). At 32 weeks after starting ruxolitinib, the percentage of patients who received at least 1 phlebotomy was significantly decreased compared with before ruxolitinib (37% vs. 56%; relative risk [RR], 0.66; 95% confidence interval [CI], 0.52-0.84; P < .001). Phlebotomy requirements were similarly decreased in patients who had received at least 3 phlebotomies prior to ruxolitinib initiation (28% vs. 17%; RR, 1.65; 95% CI, 1.13-2.40; P < .01). Resolution of palpable splenomegaly was also documented (48% vs. 20%; RR, 2.45; 95% CI, 1.70-3.53; P < .0001). A total of 9.5% of patients discontinued ruxolitinib owing to treatment-emergent adverse events, and 81.7% of patients were receiving ruxolitinib at last known follow-up.ConclusionThese real-world results are similar to those reported from the RESPONSE trial, although additional follow-up is necessary to assess long-term outcomes and potential for late-onset toxicity.     

Peritoneal carcinomatosis-like implants of extramedullary hematopoiesis. An insolite occurrence during splenectomy for myelofibrosis

IntroductionPrimary myelofibrosis (MF) is a myeloproliferative neoplasm that results in debilitating constitutional symptoms, splenomegaly, and cytopenias. In patients with symptomatic splenomegaly, splenectomy remains a viable treatment option for MF patients with medically refractory symptomatic splenomegaly that precludes the use of ruxolitinib.Case presentationWe present the clinical case of a patient who was admitted to our Department to perform a splenectomy in MF as a therapeutic step prior to an allogeneic stem cell transplantation (ASCT). A laparotomic splenectomy and excision of whitish wide-spread peritoneal and omental nodulations was performed. There were no operative complications and the surgery was completed with minimal blood loss. The histopathological exam revealed an extramedullary hematopoiesis in both spleen and peritoneal nodules.ConclusionIn primary myelofibrosis it must always be kept in mind the possible presence of peritoneal implants of extramedullary hematopoiesis and ascites of reactive genesis. We report a rare case of peritoneal carcinomatosis-like implants of extramedullary hematopoiesis found at splenectomy for MF.     

Emergency splenectomy for trauma in the setting of splenomegaly,axillary lymphadenopathy,and incidental B-cell chronic lymphocytic leukemia: A case report

IntroductionThe spleen is the most commonly injured intra-abdominal solid organ following blunt trauma. B-cell chronic lymphocytic leukemia (CLL) is the most common leukocytic dyscrasia affecting adults in Western countries. Splenomegaly with axillary and retroperitoneal lymphadenopathy are common physical findings. This case investigates an emergency splenectomy in a community hospital involving a 45-year-old man with blunt abdominal trauma following an assault with incidental splenomegaly and axillary lymphadenopathy, with surgical pathology findings of B-cell CLL.Presentation of caseA 45- year-old man without past medical or family history who was the victim of an assault presented to the emergency department 6 h later with left upper quadrant pain and radiation to the left flank and a positive Kehr sign. An elevated absolute lymphocyte count above 7 × 10⁹ and CT confirmation of a Grade V splenic laceration with splenomegaly, axillary lymphadenopathy, with hemodynamic compromise led to an exploratory laparotomy and emergency splenectomy regardless of the potential for malignancy.DiscussionHemoperitoneum with blunt splenic injury (BSI) caused by abdominal trauma with hemodynamic instability should be treated with exploratory laparotomy and splenectomy even in the face of potential malignancy with splenomegaly and axillary lymphadenopathy. An appropriate oncologic work up and treatment can be provided after the emergency intervention.ConclusionAn emergency splenectomy is an appropriate operative intervention for a grade V splenic laceration with hemoperitoneum, splenomegaly, and axillary lymphadenopathy regardless of the potential for a neoplastic process such as B-cell CLL. Post-splenectomy vaccinations and oncologic follow-up for systemic chemotherapy should be facilitated after recovery.     

A Case of Liver Fibrosis with Splenomegaly after Oxaliplatin-Based Adjuvant Chemotherapy for Colon Cancer

Previous studies reported that oxaliplatin is associated with sinusoidal obstruction syndrome. However few reports on oxaliplatin induced liver fibrosis are found in the literature. Furthermore pathogenesis of liver fibrosis is not well known. We report a case of 45-yr-old Korean man in whom liver fibrosis with splenomegaly developed after 12 cycles of oxaliplatin based adjuvant chemotherapy for colon cancer (T4N2M0). Thorough history taking and serological examination revealed no evidence of chronic liver disease. Restaging CT scans demonstrated a good response to chemotherapy. Five month after chemotherapy, he underwent right hepatectomy due to isolated metastatic lesion. The liver parenchyma showed diffuse sinusoidal dilatation and centrilobular vein fibrosis with necrosis without steatosis. We could conclude that splenomegaly was due to perisinusoidal liver fibrosis and liver cell necrosis induced portal hypertension by oxaliplatin. In addition, to investigate the pathogenesis of liver fibrosis, immunohistochemical stains such as CD31 and α-smooth muscle actin (α-SMA) were conducted with control group. The immunohistochemical stains for CD31 and α-SMA were positive along the sinusoidal space in the patient, while negative in the control group. Chemotherapy with oxaliplatin induces liver fibrosis which should be kept in mind as a serious complication.     

Littoral cell angioma of the spleen treated by laparoscopic splenectomy.

Massive splenomegaly due to multifocal littoral cell angioma was discovered incidentally in a 55-year-old man during a workup for an unrelated condition. The tumor was removed successfully by laparoscopic splenectomy. We report the second case of littoral cell angioma of the spleen treated laparoscopically.     

Niemann-Pick disease type C: nodular splenomegaly

Niemann-Pick type C disease (NPCD) is an autosomal recessive storage lipidosis due to a disorder of cholesterol esterification leading to the accumulation of sphingomyelin and cholesterol in the brain, liver, and spleen. The disease is usually diagnosed when neurological symptoms appear. We report an unusual presentation of NPCD in a young asymptomatic adult with isolated nodular splenomegaly.     

Assessment of abdominal involvement of adult T-cell leukemia/lymphoma by ultrasonography: comparison among four clinical types.

Adult T-cell leukemia/lymphoma (ATLL) is an HTLV-I associated lymphoid malignancy frequently seen in Japan. Abdominal involvement in 40 patients with ATLL were assessed by ultrasonography and the findings seen in four clinical types, acute, chronic, lymphoma and smoldering, were compared. Splenomegaly was frequently found in the cases of acute and lymphoma types, and the sizes of the spleens measured by ultrasonography correlated well with the disease activity. Hepatomegaly was also found more frequently in acute and lymphoma types, and hepatosplenomegaly was proved to be due to the infiltration by ATL cells. Nodular lesions in spleen and liver and abdominal lymph node swelling were also found frequently in the lymphoma type but rarely in the other types. Ascites, pleural effusion, and pericardial effusion were found in the active stage of acute and lymphoma types. Ultransonography also could detect findings associated with therapies. Thus, ultrasonography studies were found to be very useful for assessing the clinical classification, examining various pathological conditions associated with ATLL, and monitoring the disease activity.     

脾切除术治疗不明原因发热伴脾肿大35例临床分析

目的探讨脾切除术在不明原因发热(FUO)中的诊断价值。方法回顾性分析2001年1月至2005年6月北京协和医院的35例因FUO行诊断性脾切除术的患者的临床特点。结果35例患者中,男20例,女15例,平均年龄为39岁。所有病例均有不同程度的脾大。从发病到脾切除术的平均时间为15.8(5～165)周。经脾切除术直接诊断淋巴瘤27例、播散性结核病2例。间接诊断成人Still病4例;2例未确诊,总诊断率为94.1%。手术并发症的总发生率为14.3%,无一例因手术死亡。结论脾切除术是诊断FUO伴脾大的重要方法,选择合适的患者和手术时机是脾切除术在FUO诊断应用中的关键。