ACUTE MYELOID LEUKEMIA DIAGNOSED WITH CUTANEOUS INVOLVEMENT; A RARE CASE

HATICE AYAG , Müzeyyen Aslaner Ak , Birsen Sahip Yesiralioğlu , Pelin Ertop Doğan , Şehmus Ertop
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While the extramedullary collection of leukemic cells is generally regarded as myeloid sarcoma (previously chloroma/granulocytic sarcoma), leukemia cutis is a generic term to describe specific cutaneous involvement. Although any subtype of leukemia can involve the skin, the most common types seen in clinical practice are chronic lymphocytic leukemia (CLL) and acute myeloid leukemia (AML) with monocytic or myelomonocytic morphology(4). We present a case diagnosed with Extramedullary AML with skin involvement, but without bone marrow involvement.</p></div><div><h3>Case report</h3><p>Case: A 60-year-old female patient who presented to the dermatology outpatient clinic in March 2023 due to painful lesions on the trunk for the past 3 months. Physical examination revealed widespread palpable firm nodular lesions on the trunk and back(figüre-1).</p></div><div><h3>Methodology</h3><p>The patient underwent a punch biopsy with differential diagnoses including eosinophilic angiomatous hyperplasia, cutaneous metastasis, lupus tumidus panniculitis, T/B-cell lymphoma. CD68, Lysozyme, CD 33, CD16, CD123, TCL-1, TdT were investigated as antibodies.Immunohistochemical examination revealed widespread positivity for lysozyme, CD68, and faint diffuse CD33 in infiltrative cells. CD16, TdT, CD123, TCL-1 were negative. Histopathological diagnosis suggests compatibility with myeloid sarcoma characterized by blast cells with myelomonocytic features, demonstrating infiltration of immature atypical hemolymphoid cells in the skin and subcutaneous biopsy material. The patient was referred to our clinic due to compatibility with myeloid sarcoma and extramedullary myeloid leukemia. Initial tests during admission showed:WBC: 3.6 10^3/µL, HGB: 11.2 g/dL, PLT: 215 10^3/µL, NE: 2.3 10^3/µL, EO: 0.1 10^3/µL, BA: 0.0 10^3/µL, LDH: 297 U/L, with other biochemical values within normal range.In the bone marrow biopsy pathology of the patient revealed increased cellularity in the bone marrow elements, grade 1 increase in reticulin and reticular fibers,positive CD34 in vascular structures, blast cell ratio of 2-3%, mild increase and aggregation of megakaryocytes with CD61, decrease in myeloid series with MPO, and increase in erythroid cell islands with Glycophorin A. Flow cytometry showed 4.6% blast cells. The cytogenetic evaluation of the patient resulted in FLT3 negative, t (15, 17), (q22, q21) PML/RARA negative. The patient received ARA-C+Mitoxantrone (7+3) induction chemotherapy for extramedullary AML and recovered from neutropenia on the 18th day of treatment. 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引用次数: 0

Abstract

Objective

Acute myeloid leukemia (AML) is a heterogeneous hematologic malignancy characterized by clonal expansion of myeloid blasts in peripheral blood, bone marrow, and/or other tissues. It is the most common type of acute leukemia in adults with an age-adjusted incidence of 3.6/100,000 in the population (1). Extramedullary leukemia (EM AML), also known as myeloid sarcoma, is a rare manifestation of acute myeloid leukemia and is usually accompanied by bone marrow involvement (2). Leukemia cutis characteristically demonstrates the infiltration of the skin by neoplastic leukocytes(3). While the extramedullary collection of leukemic cells is generally regarded as myeloid sarcoma (previously chloroma/granulocytic sarcoma), leukemia cutis is a generic term to describe specific cutaneous involvement. Although any subtype of leukemia can involve the skin, the most common types seen in clinical practice are chronic lymphocytic leukemia (CLL) and acute myeloid leukemia (AML) with monocytic or myelomonocytic morphology(4). We present a case diagnosed with Extramedullary AML with skin involvement, but without bone marrow involvement.

Case report

Case: A 60-year-old female patient who presented to the dermatology outpatient clinic in March 2023 due to painful lesions on the trunk for the past 3 months. Physical examination revealed widespread palpable firm nodular lesions on the trunk and back(figüre-1).

Methodology

The patient underwent a punch biopsy with differential diagnoses including eosinophilic angiomatous hyperplasia, cutaneous metastasis, lupus tumidus panniculitis, T/B-cell lymphoma. CD68, Lysozyme, CD 33, CD16, CD123, TCL-1, TdT were investigated as antibodies.Immunohistochemical examination revealed widespread positivity for lysozyme, CD68, and faint diffuse CD33 in infiltrative cells. CD16, TdT, CD123, TCL-1 were negative. Histopathological diagnosis suggests compatibility with myeloid sarcoma characterized by blast cells with myelomonocytic features, demonstrating infiltration of immature atypical hemolymphoid cells in the skin and subcutaneous biopsy material. The patient was referred to our clinic due to compatibility with myeloid sarcoma and extramedullary myeloid leukemia. Initial tests during admission showed:WBC: 3.6 10^3/µL, HGB: 11.2 g/dL, PLT: 215 10^3/µL, NE: 2.3 10^3/µL, EO: 0.1 10^3/µL, BA: 0.0 10^3/µL, LDH: 297 U/L, with other biochemical values within normal range.In the bone marrow biopsy pathology of the patient revealed increased cellularity in the bone marrow elements, grade 1 increase in reticulin and reticular fibers,positive CD34 in vascular structures, blast cell ratio of 2-3%, mild increase and aggregation of megakaryocytes with CD61, decrease in myeloid series with MPO, and increase in erythroid cell islands with Glycophorin A. Flow cytometry showed 4.6% blast cells. The cytogenetic evaluation of the patient resulted in FLT3 negative, t (15, 17), (q22, q21) PML/RARA negative. The patient received ARA-C+Mitoxantrone (7+3) induction chemotherapy for extramedullary AML and recovered from neutropenia on the 18th day of treatment. Subsequent evaluations showed near-complete improvement (figüre-2).

Results

After the patient's discharge, BMT was planned. However, the patient was excluded at the center where they applied for BMT

Conclusion

A variant of extramedullary leukemia is leukemic skin involvement. This condition may or may not be accompanied by bone marrow involvement. The case presented here is a rare instance of Leukemia Cutis without bone marrow involvement. The patient received a myeloid leukemia treatment protocol, and significant regression was observed in skin lesions after treatment. However, our patient was excluded prior to BMT."

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急性髓性白血病诊断为皮肤受累;一个罕见病例
目的急性髓性白血病(AML)是一种异质性血液系统恶性肿瘤,其特征是外周血、骨髓和/或其他组织中髓性白细胞的克隆性扩增。它是成人中最常见的急性白血病类型,经年龄调整后的发病率为 3.6/100,000(1)。髓外白血病(EM AML)又称髓样肉瘤,是急性髓性白血病的一种罕见表现,通常伴有骨髓受累(2)。皮肤白血病的特征是肿瘤性白细胞浸润皮肤(3)。髓外聚集的白血病细胞一般被视为髓样肉瘤(以前称为绿瘤/粒细胞肉瘤),而皮肤白血病则是描述特定皮肤受累情况的通用术语。虽然任何亚型的白血病都可能累及皮肤,但临床上最常见的类型是单核细胞或髓单核细胞形态的慢性淋巴细胞白血病(CLL)和急性髓细胞白血病(AML)(4)。我们报告了一例诊断为髓外型急性髓细胞白血病的病例,患者皮肤受累,但无骨髓受累:患者女性,60 岁,因过去 3 个月躯干出现疼痛性皮损于 2023 年 3 月到皮肤科门诊就诊。体格检查显示,患者躯干和背部有广泛的可触及的坚实结节性病变(图-1)。方法:患者接受了打孔活检,鉴别诊断包括嗜酸性粒细胞血管瘤样增生、皮肤转移、狼疮瘤样泛发性炎、T/B细胞淋巴瘤。CD68、溶菌酶、CD33、CD16、CD123、TCL-1、TdT抗体均被检测。免疫组化检查显示溶菌酶、CD68广泛阳性,浸润细胞中CD33呈微弱弥漫性阳性。CD16、TdT、CD123和TCL-1呈阴性。组织病理诊断表明,该病与骨髓肉瘤相容,其特征是具有骨髓单核细胞特征的爆破细胞,皮肤和皮下活检材料显示有未成熟的非典型血淋巴细胞浸润。患者因同时患有骨髓肉瘤和髓外骨髓性白血病而被转诊至我院。入院时的初步检查结果显示:白细胞:3.6 10^3/μL,HGB:11.2 g/dL,PLT:215 10^3/μL,NE:2.3 10^3/μL,EO:0.1 10^3/μL,BA:0.0 10^3/μL,LDH:297 U/L,其他生化指标均在正常范围内。患者的骨髓活检病理结果显示,骨髓成分中细胞增多,网状纤维和网状纤维 1 级增高,血管结构中 CD34 阳性,爆破细胞比例为 2-3%,巨核细胞 CD61 轻度增高和聚集,骨髓系列 MPO 降低,红细胞岛 Glycophorin A 增高。患者的细胞遗传学评估结果为 FLT3 阴性、t(15,17)、(q22,q21)PML/RARA 阴性。患者接受了髓外急性髓细胞白血病 ARA-C+ 米托蒽醌(7+3)诱导化疗,并在治疗的第 18 天从中性粒细胞减少症中恢复过来。患者出院后,计划进行 BMT。结论 髓外白血病的一种变异型是白血病性皮肤受累。这种情况可能伴有骨髓受累,也可能不伴有骨髓受累。这里介绍的病例是一种罕见的无骨髓受累的切缘白血病。患者接受了骨髓性白血病治疗方案,治疗后皮肤病变明显消退。然而,我们的患者在接受 BMT 治疗前已被排除在外"。
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CiteScore
2.40
自引率
4.80%
发文量
1419
审稿时长
30 weeks
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