PDF(Pubmed)
Abstract：
UNASSIGNED: Dual hematological malignancies, asynchronous or synchronous, are underrecognized entities and are usually suspected when clinical, hematological, or biochemical features cannot be explained by the primary malignancy alone. We present a case of synchronous dual hematological malignancies (SDHMs), where the patient was diagnosed with symptomatic multiple myeloma (MM) and essential thrombocythemia (ET), when excessive thrombocytosis occurred following initiation of MPV (melphalan-prednisone-bortezomib) antimyeloma therapy.
UNASSIGNED: An 86-year-old woman presented to the emergency in May 2016 with confusion, hypercalcemia, and acute kidney injury. She was diagnosed with free light chain (FLC) lambda and Immunoglobulin G (IgG) lambda MM and started MPV (standard of care at that time) treatment with darbopoietin support. At diagnosis, she had normal platelet count since the ET was likely masked by bone marrow suppression due to active MM. After she reached stringent complete remission with no MP detected on serum protein electrophoresis or immunofixation, we noticed that her platelet counts increased to 1,518,000 × 109/L. She was tested positive for mutation in exon 9 of calreticulin (CALR). We concluded that she had concomitant CALR-positive ET. After bone marrow recovery from MM, the ET became clinically apparent. We started hydroxyurea for ET. Treatment for MM with MPV did not affect the course of ET. Presence of concomitant ET did not decrease the efficacy of sequential antimyeloma therapies in our elderly and frail patient.
UNASSIGNED: The possible mechanism underlying the occurrence of SDHMs is unclear but is likely due to stem cell differentiation defects. SDHMs can be challenging to treat and warrant several considerations. In the absence of clear guidelines on how to manage SDHMs, management decisions depend on several factors including disease aggressiveness, age, frailty, and comorbidities.
UNASSIGNED: An 86-year-old woman presented to the emergency in May 2016 with confusion, hypercalcemia, and acute kidney injury. She was diagnosed with free light chain (FLC) lambda and Immunoglobulin G (IgG) lambda MM and started MPV (standard of care at that time) treatment with darbopoietin support. At diagnosis, she had normal platelet count since the ET was likely masked by bone marrow suppression due to active MM. After she reached stringent complete remission with no MP detected on serum protein electrophoresis or immunofixation, we noticed that her platelet counts increased to 1,518,000 × 109/L. She was tested positive for mutation in exon 9 of calreticulin (CALR). We concluded that she had concomitant CALR-positive ET. After bone marrow recovery from MM, the ET became clinically apparent. We started hydroxyurea for ET. Treatment for MM with MPV did not affect the course of ET. Presence of concomitant ET did not decrease the efficacy of sequential antimyeloma therapies in our elderly and frail patient.
UNASSIGNED: The possible mechanism underlying the occurrence of SDHMs is unclear but is likely due to stem cell differentiation defects. SDHMs can be challenging to treat and warrant several considerations. In the absence of clear guidelines on how to manage SDHMs, management decisions depend on several factors including disease aggressiveness, age, frailty, and comorbidities.
摘要：
■双重血液恶性肿瘤，异步或同步，是未被认可的实体，通常在临床时被怀疑，血液学,或生化特征不能仅由原发性恶性肿瘤来解释。我们介绍了一例同步双重血液恶性肿瘤（SDHM），患者被诊断为有症状的多发性骨髓瘤（MM）和原发性血小板增多症（ET），当开始MPV（美法仑-泼尼松-硼替佐米）抗骨髓瘤治疗后发生过度血小板增多时。
■2016年5月，一名86岁的妇女困惑地出现在紧急情况下，高钙血症,和急性肾损伤。她被诊断为游离轻链（FLC）λ和免疫球蛋白G（IgG）λMM，并在darbopoietin支持下开始MPV（当时的护理标准）治疗。诊断时,她的血小板计数正常，因为ET可能被活动性MM引起的骨髓抑制所掩盖。在达到严格完全缓解后，血清蛋白电泳或免疫固定均未检测到MP，我们注意到她的血小板计数增加到1,518,000×109/L。她的钙网蛋白（CALR）外显子9的突变检测呈阳性。我们的结论是她同时患有CALR阳性ET。从MM中恢复骨髓后，ET在临床上变得明显。我们开始用于ET的羟基脲。MPV治疗MM不影响ET的病程。在我们的老年和体弱患者中，伴随的ET的存在并没有降低序贯抗骨髓瘤治疗的疗效。
■SDHM发生的可能机制尚不清楚，但可能是由于干细胞分化缺陷。SDHM可能具有挑战性，需要考虑几个因素。在缺乏关于如何管理SDHM的明确指导方针的情况下，管理决策取决于几个因素，包括疾病侵袭性，年龄,脆弱,和合并症。
■2016年5月，一名86岁的妇女困惑地出现在紧急情况下，高钙血症,和急性肾损伤。她被诊断为游离轻链（FLC）λ和免疫球蛋白G（IgG）λMM，并在darbopoietin支持下开始MPV（当时的护理标准）治疗。诊断时,她的血小板计数正常，因为ET可能被活动性MM引起的骨髓抑制所掩盖。在达到严格完全缓解后，血清蛋白电泳或免疫固定均未检测到MP，我们注意到她的血小板计数增加到1,518,000×109/L。她的钙网蛋白（CALR）外显子9的突变检测呈阳性。我们的结论是她同时患有CALR阳性ET。从MM中恢复骨髓后，ET在临床上变得明显。我们开始用于ET的羟基脲。MPV治疗MM不影响ET的病程。在我们的老年和体弱患者中，伴随的ET的存在并没有降低序贯抗骨髓瘤治疗的疗效。
■SDHM发生的可能机制尚不清楚，但可能是由于干细胞分化缺陷。SDHM可能具有挑战性，需要考虑几个因素。在缺乏关于如何管理SDHM的明确指导方针的情况下，管理决策取决于几个因素，包括疾病侵袭性，年龄,脆弱,和合并症。
