An Unlikely Rapid Transformation of Myelodysplastic Syndrome to Acute Leukemia: A Case Report

Pourmoussa, Andrew; Kwan, Karen

doi:10.7812/tpp/16-091

Cited by 3 publications

(6 citation statements)

References 11 publications

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“…7 Pourmoussa and Kwan reported another case of an extremely rapid transformation from MS with concomitant SS to AML, in an elderly patient. 8 Our patient also followed that sequence with a fatal ending. Myelodysplastic Syndrome can evolve into AML, which often leads to a poor prognosis.…”

Section: Discussionmentioning

confidence: 56%

Transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing sweet syndrome

et al. 2021

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The Sweet’s syndrome, is an inflammatory skin disorder characterized by extensive infiltration of neutrophils in the dermis with extension to the subcutis, known as acute febrile neutrophilic dermatosis. It may occur as a paraneoplastic syndrome. To our knowledge, there are currently few reports about transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing Sweet syndrome in the literature. Herein we describe an unusual case in a young patient with these characteristics that evolved to a fatal outcome.

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Section: Discussionmentioning

confidence: 56%

Transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing sweet syndrome

et al. 2021

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“…Deletion of the long arm of chromosome 5 (del (5q)) remains one of the most frequent abnormalities associated with MDS [ 4 , 5 ]. While often associated with a good prognosis and infrequently with transformation to leukemia, our patient was noted to have a phenotype three months prior consistent with less than 5% blasts.…”

Section: Discussionmentioning

confidence: 99%

Clostridium difficile Infection Complicated by Transformation of Myelodysplastic Syndrome to Acute Myeloid Leukemia With Leukostasis and Sweet’s Syndrome

Landry

Vest

Williams

2021

Cureus

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Myelodysplastic syndrome (MDS) is a premalignant condition characterized by clonal proliferation and ineffective hematopoiesis. The subtype of MDS associated with deletion in the long arm of chromosome 5 is generally associated with older females and carries a good prognosis as it rarely transforms to acute myeloid leukemia. The mechanisms of leukemic transformation are still poorly understood and likely involve a variety of somatic mutations and epigenetic modifications. We present the case of a 70-year-old female with known MDS with deletion 5(q) who presented with anemia, thrombocytopenia, and guaiac positive stool who was subsequently found to be positive for Clostridium difficile infection. During the course of her treatment, she developed significant leukocytosis, splenic infarction, and acute hypoxic respiratory failure requiring high flow nasal cannula. Flow cytometry returned positive for increased blasts of more than 30%. She was transferred to a tertiary care facility for cytoreductive therapy and developed leukostasis and Sweet's syndrome.

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“…1-3 Risiko transformasi leukemia ditentukan sebagian oleh tingkat atipia dari morfologi, persentase blast di sumsum tulang, dan sitogenetika MDS. 4 MDS merupakan penyakit yang sering ditemukan pada pasien lanjut usia. Usia rerata saat onset penyakit sekitar 70 tahun.…”

Section: Pendahuluanunclassified

“…Manifestasi penyakit yang khas termasuk kelelahan dan kelemahan yang disebabkan oleh anemia, infeksi yang disebabkan oleh neutropenia, atau perdarahan yang disebabkan oleh trombositopenia atau disfungsi trombosit. 4 Berbagai manifestasi penyakit termasuk perubahan persisten pada garis sel yang menyebabkan komplikasinya masingmasing, dan sindrom paraneoplastik. 5 Diagnosis MDS ditegakkan berdasarkan pemeriksaan hematologi, morfologi sel darah tepi dan sumsum tulang yang menunjukkan sitopenia pada satu atau lebih garis hematopoetik atau displasia pada hapusan darah, sebagian besar pasien anak lebih sering ditemukan dengan pansitopenia, bukan hanya anemia (sumsum tulang hiposelular), pemeriksaan lanjutan sitogenetika serta immunophenotyping, namun dua pemeriksaan terakhir ini tidak tersedia di semua rumah sakit di Indonesia sehingga diagnosis MDS terbatas pada pemeriksaan morfologi sel darah dan sumsum tulang.…”

Section: Pendahuluanunclassified

“…Manifestasi penyakit meliputi kelelahan dan kelemahan, infeksi, atau perdarahan. 4 Anamnesis dan pemeriksaan fisik MDS yaitu riwayat keluarga yang terperinci setidaknya 2 generasi sebelumnya, termasuk kanker, kegagalan sumsum tulang, gangguan hati/paru-paru atau kematian dini; kemoterapi atau riwayat radiasi sebelumnya, paparan kerja, konsumsi alkohol, atau pengobatan yang sedang dijalani; kecenderungan terjadinya perdarahan atau infeksi; pemeriksaan fisik lengkap termasuk ukuran limpa. 6 Hasil laboratorium saat perawatan hari pertama didapatkan adanya peningkatan leukosit 50,5 (4,1-11x10 3 / mL), penurunan eritrosit 2,78 (4-5,2x10 3 / mL), penurunan hemoglobin 7,5 (12-16 mg/dl), dan penurunan hematokrit 24,7 (36-46).…”

Section: Pembahasanunclassified

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Myelodysplastic syndromes: Laporan Kasus

Wijaya

2022

Intisari Sains Medis

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Background: Myelodysplastic syndrome (MDS) is a group of bone marrow disorders characterized by ineffective hematopoiesis resulting in cytopenia and an increased risk of Acute Myeloid Leukemia (AML). MDS is a disease that is often found in elderly patients. The mean age at disease onset was 70 years. Case: A 72-year-old woman complained of feeling tired easily since 5 months throughout the day, not aggravated by activity and starting to gain weight in the last 1 week to interfere with the patient's activities. The patient also complained of intermittent dizziness since 2 months, was not affected by activity or change of position, and was not accompanied by nausea and vomiting. Complaints of fever, prolonged cough, bleeding, yellow body, red skin rash, progressive weight loss, bowel and bladder disorders were denied. Physical examination of the patient revealed vital signs within normal limits. The patient's conjunctiva was anemic, the patient's extremities also looked pale and there was no organ enlargement such as hepatomegaly and splenomegaly. Laboratory examinations at the time of presentation showed an increase in leukocytes, a decrease in erythrocytes, a decrease in hemoglobin, and a decrease in hematocrit. The patient's platelets were still in the normal range and there were no disturbances in kidney and liver function. The results of leukocytes, erythrocytes, and hemoglobin taken consecutively on 3 consecutive days still show an increase in leukocytes despite the downward trend in value. The results of bone marrow examination, namely trephine morphology, showed hypercellular marrow (approximately 80% cellularity) with an increase in blast cells of 10-15%, decreased maturation of myeloid series, erythroid cells and dyserythropoiesis, scattered plasma cells, increased megakaryocytes accompanied by dysmegakaryopoiesis. Patient diagnosed as Myelodysplastic Syndrome with Excess Blast. The therapy given to the patient during hospitalization aims to overcome the symptoms experienced by the patient. Transfusion of PRC (Packed Red Cell) 1 Kolf/day with Furosemide 20 mg by injection before transfusion and administration of Folic Acid 1 x 1 tablet per day provided an improvement in the patient's condition. This is evidenced by the increase in hemoglobin levels during treatment. The patient was referred to a referral hospital for further treatment. Conclusion: Myelodysplastic syndrome (MDS) is a group of bone marrow disorders characterized by ineffective hematopoiesis resulting in cytopenia and an increased risk of acute myeloid leukemia (AML). The patient was diagnosed with MDS with Excess Blast based on bone marrow biopsy. Other investigations, namely complete blood count, found an increase in leukocytes and hemoglobin, this also supports the diagnosis of MDS in this patient. The prognosis in this patient is classified as high risk based on IPSS-R, which means survival for 1-2 years is 13%. Latar Belakang: Myelodysplastic syndrome (MDS) adalah kelompok penyakit gangguan sumsum tulang ditandai dengan hematopoiesis yang tidak efektif mengakibatkan sitopenia dan peningkatan risiko terjadinya Leukemia Mieloid Akut (AML). MDS merupakan penyakit yang sering ditemukan pada pasien lanjut usia dengan rerata onset penyakit yaitu usia 70 tahun. Kasus: Perempuan berusia 72 tahun mengeluh mudah lelah sejak 5 bulan yang dirasakan sepanjang hari, tidak diperberat dengan aktivitas dan mulai bertambah berat 1 minggu terakhir hingga menganggu aktivitas pasien. Keluhan pusing yang hilang timbul sejak 2 bulan, tidak dipengaruhi oleh aktivitas atau perubahan posisi, dan tidak disertai dengan mual dan muntah. Keluhan demam, batuk lama, perdarahan, badan kuning, ruam merah dikulit, penurunan berat badan yang progresif, gangguan BAB dan BAK disangkal. Pasien didapatkan tanda vital dalam batas normal. Konjungtiva pasien anemis, ekstremitas pasien juga tampak pucat dan tidak didapatkan pembesaran organ seperti hepatomegaly dan splenomegaly. Hasil laboratorium diperoleh leukositosis, eritropenia, anemia, dan penurunan hematokrit. Platelet pasien masih berada pada rentang normal dan tidak terdapat gangguan pada fungsi ginjal dan hati. Hasil leukosit, eritrosit, dan hemoglobin yang diambil berturut-turut pada 3 hari berturut-turut tetap menunjukkan peningkatan leukosit meskipun tren nilai mengalami penurunan. Hasil pemeriksaan sumsum tulang yaitu gambaran morfologi trephine menunjukkan sumsum hiperseluler (kurang lebih 80% selularitas) dengan peningkatan sel blast 10 – 15 %, penurunan maturasi seri myeloid, sel eritroid dan diseritropoiesis, scattered sel plasma, peningkatan megakariosit disertai dismegakariopoiesis. Pasien diagnosa sebagai Myelodysplastic Syndrome with Excess Blast. Terapi yang diberikan pada pasien selama perawatan di rumah sakit bertujuan untuk mengatasi gejala yang dialami pasien. Transfusi PRC (Packed Red Cell) 1 Kolf/hari dengan pemberian Furosemide 20 mg secara injeksi sebelum transfusi dan pemberian Asam Folat 1 x 1 tablet per hari memberikan perbaikan kondisi pada pasien. Hal ini dibuktikan dengan peningkatan kadar hemoglobin selama perawatan. Pasien dirujuk ke rumah sakit rujukan untuk penanganan lebih lanjut. Simpulan: Myelodysplastic syndrome (MDS) adalah kelompok penyakit gangguan sumsum tulang ditandai dengan hematopoiesis yang tidak efektif mengakibatkan sitopenia dan peningkatan risiko terjadinya Leukemia Myeloid Akut (AML). Pasien didiagnosis mengalami MDS with Excess Blast berdasrakan pemeriksaan biopsis sumsum tulang. Prognosis pada pasien ini tergolong high risk berdasarkan IPSS-R yang artinya survival untuk 1 – 2 tahun yaitu 13%.

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An Unlikely Rapid Transformation of Myelodysplastic Syndrome to Acute Leukemia: A Case Report

Cited by 3 publications

References 11 publications

Transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing sweet syndrome

Transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing sweet syndrome

Clostridium difficile Infection Complicated by Transformation of Myelodysplastic Syndrome to Acute Myeloid Leukemia With Leukostasis and Sweet’s Syndrome

Myelodysplastic syndromes: Laporan Kasus

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