ArticlePDF Available

Peningkatan profil klinik-hematologi pada pasien sindrom mielodisplasia multilini paska splenektomi total

June 2020
Jurnal Penyakit Dalam Udayana 4(1):1-4

DOI:10.36216/jpd.v4i1.133

License
CC BY 4.0

Authors:

Adeodatus yuda Handaya

Universitas Gadjah Mada

Ibnu Purwanto

Universitas Gadjah Mada

Myelodysplasia syndrome (MDS) is a hematologic disorder manifested by cytopenia and rarely with splenomegaly. Clinical complaints that often accompany symptoms of anemia and when experiencing splenomegaly, the patient feels a full stomach or sometimes pain in the upper left abdomen. We report a case of a 55 year old woman with a full stomach complaint with a history of thrombocytopenia. From the peripheral blood examination, observation of thrombocytopenia was obtained with suspicion of the inflammatory process and physical examination of splenomegaly. Bone marrow aspiration studies revealed a multi-line cytopenic refractory MDS. Patients receive immunosuppressant therapy without blood transfusions. After 1 year of treatment the patient complained of fatigue and upper left abdominal pain. The results showed pancytopenia and splenomegaly. The doctor and patient discussion agreed to the splenectomy. The patient had no complications after splenectomy and for 11 months the patient felt complaint free and normal hematological parameters with a good quality of life. This case report suggests that splenectomy can be an alternative therapy for MDS that is refractory to previous therapy and is complaining of splenomegaly. Splenectomy can improve the clinical complaints and hematologic profile of MDS patients with splenomegaly.

Available via license: CC BY 4.0

Content may be subject to copyright.

Open Access: www.jpdunud.org 1

ARTIKEL ASLI

Print ISSN: 2580-2925

Online ISSN: 2580-2933

Jurnal Penyakit Dalam Udayana

Udayana Journal of Internal Medicine

Volume 4, No 1: 2020 ; 1-4

Siswi Oktariani1, Adeodatus Yuda Handaya2, Ibnu Purwanto3*

Tanggal diterima : 12 Februari 2020

Tanggal Disetujui : 12 Maret 2020

Tanggal Diterbitkan : 20 Juni 2020

1Trainee, Divisi Hematologi dan

Onkologi Medis, Departemen

Ilmu Penyakit Dalam, Fakultas

Kedokteran, Kesehatan Masyarakat

dan Keperawatan, Universitas Gadjah

Mada/ Rumah Sakit Dr.Sardjito,

Yogyakarta, Indonesia.

2Divisi Bedah Digestif, Bagian

Ilmu Penyakit Bedah, Fakultas

Kedokteran, Kesehatan Masyarakat

dan Keperawatan, Universitas Gadjah

Mada/ Rumah Sakit Dr.Sardjito,

Yogyakarta, Indonesia.

3Divisi Hematologi dan Onkologi Medik,

Bagian Ilmu Penyakit Dalam, Fakultas

Kedokteran, Kesehatan Masyarakat

dan Keperawatan, Universitas Gadjah

Mada/ Rumah Sakit Dr.Sardjito,

Yogyakarta, Indonesia.

*Korespondensi:

Ibnu Purwanto; Divisi Hematologi dan

Onkologi Medik, Bagian Ilmu Penyakit

Dalam, Fakultas Kedokteran, Kesehatan

Masyarakat dan Keperawatan,

Universitas Gadjah Mada/ Rumah Sakit

Dr.Sardjito, Yogyakarta, Indonesia;

ibnu.purwanto.md@gmail.com

Myelodysplasia syndrome (MDS) is a hematologic disorder manifested by cytopenia and rarely with splenomegaly.

Clinical complaints that often accompany is symptoms of anemia and when experiencing splenomegaly, the patient

feels a full stomach or sometimes pain in the upper left abdomen. We report a case of a 55 year old woman with a

full stomach complaint with a history of thrombocytopenia. From the peripheral blood examination, observation of

thrombocytopenia was obtained with suspicion of the inammatory process and physical examination of splenomegaly.

Bone marrow aspiration studies revealed a multi-line cytopenic refractory MDS. Patients receive immunosuppressant

therapy without blood transfusions. After 1 year of treatment the patient complained of fatigue and upper left

abdominal pain. The results showed pancytopenia and splenomegaly. The doctor and patient discussion agreed to the

splenectomy. The patient had no complications after splenectomy and for 11 months the patient felt complaint free

and normal hematological parameters with a good quality of life. This case report suggests that splenectomy can be an

alternative therapy for MDS that is refractor y to previous therapy and is complaining of splenomegaly. Splenectomy can

improve the clinical complaints and hematologic prole of MDS patients with splenomegaly.

Keyword: myelodysplasia syndrome-splenomegali-splenektomi

Myelodysplasia syndrome (MDS) merupakan kelainan hematologi dengan manifestasi sitopenia dan jarang disertai

splenomegali. Keluhan klinis yang sering menyertai adalah simtom anemia dan bila mengalami splenomegali pasien

merasa perut terasa penuh atau kadang nyeri di perut kiri atas. Kami melaporkan kasus wanita 55 tahun dengan

keluhan perut terasa penuh disertai riwayat trombositopenia. Dari pemeriksaan darah tepi didapatkan observasi

trombositopenia dengan curiga proses inamasi dan pemeriksaan sik splenomegali. Pemeriksaan aspirasi sumsum

tulang mendapatkan suatu MDS refrakter sitopenia multi lini. Pasien mendapatkan terapi imunosupresan tanpa

transfusi darah. Setelah 1 tahun menjalani pengobatan pasien mengeluh mudah lelah dan nyeri perut kiri atas.

Hasil pemeriksaan mendapatkan pansitopenia dan splenomegali. Diskusi dokter dan pasien menyetujui tindakan

splenektomi. Pasien tidak mengalami komplikasi paska splenektomi dan selama 11 bulan pasien merasa bebas keluhan

serta parameter hematologi yang normal dengan kualitas hidup yang baik. Laporan kasus ini menunjukkan bahwa

splenektomi dapat menjadi alternatif terapi bagi MDS yang refrakter terhadap terapi sebelumnya dan mengalami

keluhan akibat splenomegali. Splenektomi dapat memperbaiki keluhan klinis dan prol hematologi pasien MDS

dengan splenomegali.

Kata kunci: myelodysplasia syndrome-splenomegali-splenektomi

DOI : 10.36216/jpd.v4i1.133

PENDAHULUAN

Sindrom mielodispasia (MDS) adalah neoplasma grup

mieloid yang ditandai oleh sitopenia karena hematopoiesis

yang tidak efektif, morfologi darah sumsum tulang yang

abnormal dan berisiko untuk berkembang menjadi leukemia

mieloblastik aku.1 Manifestasi klinis MDS sangat bervariasi

tergantung pada lini seluler yang terlibat. Meskipun proses

klonal pada MDS memberikan gambaran hiperseluler pada

sumsum tulang, namun biasanya pasien mengalami sitopenia

perifer karena apoptosis intramedular terhadap sel klonal

yang mengalami displasia.2

Infeksi, perdarahan dan anemia adalah gambaran

umum pada MDS, sedangkan temuan yang tidak biasa adalah

hepatomegali, splenomegali dan limfadenopati. Patofisiologi

splenomegali pada MDS belum dapat dijelaskan.2 Kraus

melaporkan hasil pemeriksaan histologi dan imunofenotipik

dari lien paska splenomegali dan post-mortem yang

menyimpulkan bahwa splenomegali dalam MDS terjadi akibat

sekuel/gejala sisa dari dispoiesis dan bukan menggambarkan

proliferasi.3 Menurut Shimomura splenomegali berhubungan

dengan kesintasan hidup yang buruk setelah transplantasi

Peningkatan prol klinik-hematologi pada pasien sindrom

mielodisplasia multilini paska splenektomi total

2 Open Access: www.jpdunud.org

ARTIKEL ASLI

Print ISSN: 2580-2925

Online ISSN: 2580-2933

Jurnal Penyakit Dalam Udayana

Udayana Journal of Internal Medicine

Volume 4, No 1: 2020 ; 1-4

alogenik pada pasien MDS.4 Bourgeois menyatakan bahwa

pada beberapa kasus MDS risiko rendah dengan splenomegali,

terjadi trombositopenia yang disebabkan oleh kerusakan

perifer. Pada kondisi ini, disarankan dilakukan splenektomi

untuk meningkatkan prognosisnya.5 Tulisan ini melaporkan

pasien MDS multi lini dengan splenomegali, suatu kondisi

yang jarang terjadi, dan mengalami perbaikan keluhan maupun

parameter hematologi dengan tindakan splenektomi total,

suatu tindakan yang jarang dilakukan terutama di Indonesia.

ILUSTRASI KASUS

Pada tahun 2015, seorang wanita berusia 55 tahun

datang ke klinik hematologi untuk kontrol karena diketahui

mengalami trombositopenia 90/mm3 saat menjalani operasi

hemoroidektomi 1 tahun sebelumnya. Saat datang pasien

dalam kondisi umum yang baik, tanpa manifestasi perdarahan

dan tanpa organomegali. Pemeriksaan darah menunjukkan

hemoglobin 11,3 gr/dl, leukosit 3,7/ μL, trombosit 71/ μL.

Pemeriksaan morfologi darah tepi didapatkan hasil eritrosit

normositik normokromik, lekosit dan trombosit menurun

disertai gambaran infeksi/inflamasi. Pasien dilakukan

pemeriksaan aspirasi sumsum tulang untuk melacak penyebab

bisitopenia dengan hasil didapatkan jumlah sel ketiga lini

mencukupi dimana ditemukan gambaran megakariosit dan

disgranulasi trombosit pada lini trombopoeitik, pada lini

eritropoeitik didapatkan sel eritrosit berinti (NRBC, nucleated

red blood cell) dan lini granulopoeitik ditemukan mieloblast

3%, promielosit 11%, mielosit 2%, metamielosit 12%, stab

15%, segmen 22%, monoblast 1%, promonosit 1%, monosit

4%, eosinofil 6%. Gambaran lainnya didapatkan displasia

promielosit, sel pseudopelger heuet, limfoblast 1%, dan sel

plasma 2%. Kesimpulan hasil BMP adalah MDS dengan

sitopenia refrakter multi lini. Pasien tidak mengalami keluhan

dan direncanakan monitor berkala. Pasien juga menderita

diabetes mellitus (DM) dengan pemantauan glukosa darah

terkontrol dengan terapi metformin dan sulfoniluria.

Pada 3 tahun setelah terdiagnosis MDS pasien

memeriksakan diri karena merasa mudah lelah dan perut kiri

atas membesar. Pasien menyangkal keluhan demam, gangguan

buang air kecil dan besar, perdarahan dan badan kuning.

Pasien mengalami penurunan berat badan yang ringan yaitu

1 kg dalam 1 bulan terakhir. Pemeriksaan fisik mendapatkan

splenomegali. Pemeriksaan laboratorium mendapatkan Hb

9 gr/dl, angka leukosit 3 x103/mm3 dan trombosit 65 x103/

mm3. Hitung jenis leukosit mendapatkan jumlah segmen 60%,

stab 26%, monosit 8.6%, eosinophil 4.3% dan basophil 0.7%.

Pemeriksaan USG abdomen mendapatkan splenomegali dan

mencurigai gambaran sirosis hati namun setelah dikonfirmasi

dengan pemeriksaan MRI abdomen didapatkan hasil

splenomegali dengan kondisi hati tak ada kelainan.

Pasien mendapatkan terapi siklosporin-A dengan

dosis 2x50 mg selama 1 tahun. Pemantauan hasil pemeriksaan

hematologi berkesan bisitopenia dengan Hb berkisar 8-9,5

gr/dl dan trombosit 60-90 x 103/mm3. Pasien tidak pernah

mengalami perdarahan spontan namun merasa mudah lelah

dan terganggu karena perut terasa penuh dan nyeri di perut

kiri atas. Diskusi antara dokter dengan pasien membicarakan

kemungkinan tindakan splenektomi untuk mengatasi

keluhan anemia dan perut nyeri akibat splenomegali. Pasien

menyetujui tindakan splenektomi dan dilakukan splenektomi

total.

Pasien mendapatkan transfusi darah merah sebelum

operasi dengan hasil pemeriksaan laboratorium pre operatif

adalah Hb 9.8 gr/dl, leukosit 2.7x103 /mm3 dan trombosit 54

x 103/ mm3. Pemeriksaan glukosa darah 220 gr/dl, fungsi hati

dan ginjal menunjukkan hasil normal serta serologi untuk

virus hepatitis B negatif. Profil koagulasi mendapatkan PT

19,2 detik (kontrol 13,5), INR 1,46, APTT 32,9 detik (kontrol

29,7). Pasien tidak mengalami komplikasi perdarahan dan

infeksi paska splenektomi total selama masa rawat di rumah

sakit selama 9 hari.

Pemantauan paska operasi mendapatkan selama 10

bulan paska splenektomi pasien dalam kondisi fisik baik dan

parameter hematologi normal dengan rerata Hb 11.2 gr/

dl, leukosit 5.9-6.5 x 103/ mm3, trombosit 320-398 x103/

mm3. Pasien dapat menjalankan aktifitas sehari-hari dengan

kualitas hidup yang baik. Namun pada bulan ke-11 paska

splenektomi pasien kembali mengeluhkan mudah lelah dan

hasil pemeriksaan darah menunjukkan Hb 8 gr/dl dengan

leukosit dan trombosit tetap normal.

DISKUSI

Splenektomi adalah prosedur yang umum dilakukan

pada pasien dengan berbagai gangguan hematologis. Namun

tindakan splenektomi pada MDS jarang dilakukan meskipun

terjadi sitopenia berat.3 Penelitian Bickenbach melaporkan 381

pasien yang dilakukan splenektomi, terdapat 9,7% diantaranya

dengan diagnosis MDS sedangkan diagnosis lainnya adalah

limfoma non Hodgkin (49,3%), limfoma Hodgkin (2,4%),

leukemia limfositik kronik (13,1%), leukemia mielositik

kronik (3,9%), leukemia mieloblastik akut (2,4%), leukemia

limfoblastik akut (0,5%), ITP (4,5%), anemia hemolitik (2,6%),

sferositosis herediter (0,8%) dan lain-lain (9,4%).6 Sedangkan

penelitian Rialon melaporkan 89 pasien dengan kelainan

hematologi yang menjalani splenektomi terdiri atas 8%

dengan MDS, 10% leukemia akut mieloblastik, 69% penyakit

mieloproliferatif, dan 13% dengan penyakit mielodisplastik–

mieloproliferatif.7

Lien berperan penting dalam sistem hematologi dan

imunologi. Lien sebagai bagian dari sistem retikulo-endotel

dan berperan utama dalam respons imunologis segera terhadap

antigen dalam darah. Lien bertanggung jawab untuk membuat

antibodi dan menghilangkan bakteri, sel tua, sel darah yang

dilapisi antibodi dan yang rusak. Lien terlibat dalam gangguan

hematologi melalui penghancuran eritrosit dimana secara

intrinsik eritrosit abnormal atau yang dilapisi antibodi, secara

dini akan dihancurkan oleh makrofag lien. Lien juga berfungsi

sebagai tempat berkumpulnya 30-40% trombosit dari sirkulasi

dan memainkan peran dalam regulasi volume plasma.8

Open Access: www.jpdunud.org 3

ARTIKEL ASLI

Print ISSN: 2580-2925

Online ISSN: 2580-2933

Jurnal Penyakit Dalam Udayana

Udayana Journal of Internal Medicine

Volume 4, No 1: 2020 ; 1-4

Lien yang abnormal menimbulkan dua sindrom

utama yaitu hipersplenisme dan splenomegali simtomatik.

Hipersplenisme mengacu pada anemia, neutropenia dan atau

trombositopenia yang timbul secara tunggal atau kombinasi

sebagai akibat secara langsung dari pembesaran lien apa

pun penyebabnya. Suatu penelitian retrospektif menyatakan

bahwa trombositopenia karena hipersplenisme terjadi karena

sebagian besar trombosit terisolasi dalam lien sehingga

sitopenia akan terkoreksi paska splenektomi. Penjelasan lain

menyatakan trombosit yang terperangkap dalam lien dapat

mencapai 98% dari angka total. Lien juga dapat mempersingkat

kelangsungan hidup trombosit. Sedangkan neutropenia

pada hipersplenisme sangat mungkin sebagai akibat dari

marginalisasi granulosit ke dalam pembuluh darah besar lien.8

Splenomegali simtomatik paling sering terjadi

pada gangguan limfoproliferatif dan mieloproliferatif.

Peningkatan metabolisme akibat gangguan hematologi

ini dapat menyebabkan penurunan berat badan, demam,

hiperhidrosis dan nyeri kuadran kiri atas yang mungkin

berhubungan dengan penurunan aliran darah di lien dan

menyebabkan infark.8 Suatu laporan memaparkan 94% pasien

memiliki simtom akibat splenomegali yaitu nyeri perut (63%),

perut terasa penuh (45%), rasa lemah (65%) dan berat badan

menurun (38%). Paska splenektomi sebanyak 52% pasien pada

polulasi tersebut mengalami perbaikan gejala.7

Bickenbach melaporkan bahwa indikasi melakukan

splenektomi pada kelainan hematologi adalah adanya

trombositopenia persisten, anemia refrakter, keluhan akibat

splenomegali, tujuan diagnostik, sebagai terapi primer

maupun terapi antara sehingga memungkinkan pemberian

terapi lanjutan.6 Splenektomi pada pasien dengan MDS

merupakan pilihan terapi yang sangat jarang dilakukan.

Dalam kebanyakan kasus, splenektomi diindikasikan untuk

pasien MDS dengan trombositopenia terkait imun. Analisis

data penelitian selama beberapa tahun menyimpulkan

bahwa splenektomi masih merupakan pilihan yang memadai

untuk bentuk-bentuk MDS yang berbeda, terutama bentuk

hipoplastik sumsum tulang dengan sel blas kurang dari 10%,

refrakter terhadap pengobatan imunosupresif awal, refrakter

terhadap transfusi.9 Indikasi splenektomi pada pasien ini

adalah keluhan mudah lelah akibat anemia refrakter dan rasa

penuh di perut karena splenomegali.

Penelitian di Rusia melaporkan hasil tindakan

splenektomi pada 33 pasien dengan MDS. Keluaran paska

splenektomi adalah peningkatan sitopenia yang menetap

sebesar 48%, penurunan ketergantungan transfusi 42% dan

bebas transfusi 6%.9 Pasien ini mengalami bebas keluhan

dengan kualitas hidup yang baik dan parameter hematologi

yang normal paska splenektomi selama 11 bulan. Setelahnya

pasien mengalami anemia kembali.

Persiapan pre operatif untuk splenektomi disesuaikan

dengan protokol di tiap pemberi layanan. Laporan

menyebutkan pasien mendapatkan secara rutin vaksin

Pneumococcus polivalen, Meningococcus dan Haemophilus

influenzae minimal 3 minggu sebelum splenektomi. Semua

pasien mendapatkan antibiotik profilaksis dengan penisilin

oral setelah splenektomi.7 Pasien ini tidak mendapatkan

vaksin pra operatif karena belum ada kebijakan di rumah sakit

tempat perawatan dan masalah ketersediaan vaksin. Pasien

mendapatkan antibiotik berspektrum luas selama perioperatif.

Komplikasi jangka pendek paska splenektomi

diantaranya perdarahan, tromboemboli, abses subfrenik

dan infeksi (overwhelming post splenectomy infection, OPSI).8

Laporan penelitian Rialon menyebutkan komplikasi 30 hari

paska splenektomi berdasar skor Clavien–Dindo pada 89

pasien dengan kelainan hematologi sebanyak 38%, dimana 2%

pasien mengalami infeksi luka operasi, 1% emboli pulmo, 10%

trombosis vena porta, 11% pneumonia atau infeksi saluran

kemih, 6% abses intra abdomen, 6% kebocoran drainase dan

13% mengalami perdarahan yang mengharuskan operasi

perbaikan.7 Pasien ini menjalani rawat inap di rumah sakit

saat tindakan operasi dengan masa cukup pendek dan tidak

mengalami komplikasi paska operasi.

Sebuah penelitian terbaru mengungkapkan

peningkatan risiko untuk terjadi kanker spesifik pada

pasien yang menjalani splenektomi karena non-trauma,

meskipun belum bisa mengesampingkan faktor lain seperti

penyakit yang mendasari dan kebiasaan gaya hidup seperti

merokok.8 Sebuah laporan kasus mendapatkan seorang pasien

MDS dengan anemia refrakter yang kemudian mengalami

lekositosis dan trombositosis reaktif paska splenektomi

sehingga mendapatkan hidroksi urea. Penulis ini berpendapat

bahwa splenektomi akan memberikan keluaran yang buruk

bila dilakukan pada pasien MDS dengan splenomegali tanpa

trombositopenia.10

Penelitian Rialon melaporkan kematian paska

splenektomi 30 hari mencapai 18%. Sebagian besar pasien

meninggal karena kegagalan kardiorespirasi (38%), kegagalan

organ multi sistem (31%), infeksi/sepsis (13%), sindrom

distres pernafasan akut (6%), hematom subdural (6%), dan

sebab yang tak diketahui (6%). Faktor risiko yang berkaitan

dengan kematian 30 hari meliputi anemia, trombositopenia,

albumin <3 g/dl dan komorbid kardiovaskuler. Variabel

yang menurunkan kesintasan dari analisis multivariat adalah

diagnosis MDS/MPN, anemia, angka leukosit abnormal dan

hipoalbuminemia <3 g/dl. Kesintasan hidup pasien paska

splenektomi 1 tahun, 5 tahun dan 10 tahun sebesar 44%,

21%, dan 4%. Angka median kesintasan pasien adalah 278

hari paska splenektomi.7 Penelitian Taner menyebutkan

bahwa faktor risiko penurunan kesintasan umum pasien

dengan berbagai kelainan hematologi termasuk MDS adalah

anemia dengan ketergantungan transfusi sebelum operasi dan

trombositopenia.11 Sedang penelitian Bickenbach mendapatkan

status performa yang buruk dan trombositopenia merupakan

faktor risiko yang meningkatkan mortalitas.6

RINGKASAN

Pasien MDS merupakan salah satu penyakit kelainan

hematologi yang cukup banyak. Keluhan tersering pasien

adalah terkait sitopenia dan splenomegali. Telah dilaporkan

4 Open Access: www.jpdunud.org

ARTIKEL ASLI

Print ISSN: 2580-2925

Online ISSN: 2580-2933

Jurnal Penyakit Dalam Udayana

Udayana Journal of Internal Medicine

Volume 4, No 1: 2020 ; 1-4

This work is licensed under a

Creative Commons Attribution 4.0

International License.

kasus seorang pasien MDS yang disertai dengan splenomegaly

dan trombositopenia. Pasien mendapat tindakan splenektomi.

Setelah splenektomi, pasien mengalami perbaikan keluhan

dan kondisi. Splenektomi merupakan salah satu pilihan terapi

yang dapat dipertimbangkan pada kondisi pasien refrakter

terhadap terapi sebelumnya atau karena keterbatasan pilihan

terapi medikamentosa yang ada.

DAFTAR PUSTAKA

1. Steensma DP. Myelodysplastic syndromes current treatment algorithm

2018. Blood Cancer Journal. 2018;8(47).

2. Nalluru SS., Jindal V, Piranavan P. et al. Splenic infarction secondary to

myelodysplastic syndrome: unravelling more etiologies. AME Case Rep.

2019;3:31.

3. Kraus MD. Splenic histology and histopathology: an update. Semin

Diagn Pathol. 2003;20(2):84-93.

4. Shimomura Y, Hara M, Katoh D, dkk. Enlarged spleen is associated

with low neutrophil and platelet engraftment rates and poor survival

after allogeneic stem cell transplantation in patients with acute myeloid

leukemia and myelodysplastic syndrome. Ann Hematol. 2018;97(6):1049-

1056.

5. Bourgeois E, Caulier MT, Rose C, dkk. Role of splenectomy in

the treatment of myelodysplastic syndromes with peripheral

thrombocytopenia: a report on six cases. Leukemia. 2001;15(6):950-953.

6. Bickenbach KA, Gonen M, Labow DM, dkk. Indications for and efficacy

of splenectomy for haematological disorders. Br J Surg. 2013;100:794–

800.

7. Rialon KL, Speicher PJ, Ceppa EP, dkk. Outcomes following

splenectomy in patients with myeloid neoplasms. J Surg Oncol. 2015 Mar

15;111(4):389-95

8. Weledji P. Benefits and risks of splenectomy. Int J Surg . 2014;12(2):113-

119

9. Kokhno AV, Mikhailova EA, Parovichnikova EN, dkk. Splenectomy in

Patients with MDS. Blood. 2010;116(21):1879.

10. Uchida R, Nakano S, Namura K, dkk. Is splenectomy a contraindication

for patients with myelodysplastic syndromes complicated by

splenomegaly?. Ann Hematol. 2006;85(3):198-199.

Pansitopenia: Challenges Of Approach And Case Handling In Peripheral Hospitals

Article

Full-text available

Oct 2024

Pancytopenia is a hematological condition characterized by a decrease in three blood cell lines: erythrocytes, leukocytes, and platelets. This condition is not an independent disease, but a manifestation of various underlying diseases. Pancytopenia can be caused by impaired blood cell production in the bone marrow or increased destruction of blood cells in the periphery. Precise and prompt identification of the etiology is essential, given that pancytopenia can be life-threatening if not treated properly. This study aims to analyze and identify the causes of pancytopenia in patients at Batang Regency Hospital, as well as provide recommendations for appropriate treatment based on the etiology found. The methods used were clinical observation, laboratory examination, and diagnostic imaging in a 58-year-old patient with the main complaint of weakness and a history of pancytopenia. The examination includes a complete blood count, a smear of peripheral blood, and a clinical evaluation. The results showed that the patient had central type pancytopenia, with a suspected etiology of aplastic anemia or myelodysplastic syndrome. Other differential diagnoses include vitamin B12 deficiency. Although a bone marrow biopsy is necessary to confirm the diagnosis, initial therapy with blood transfusions and nutritional improvements has shown an improvement in the patient's condition. The conclusion of this study is the importance of a thorough evaluation to identify the etiology of pancytopenia. Proper treatment should be based on the underlying cause, which will affect the prognosis and prevent recurrence. A comprehensive approach can improve patients' quality of life and reduce the risk of complications

Myelodysplastic syndromes current treatment algorithm 2018

Article

Full-text available

May 2018

David Steensma

Myelodysplastic syndromes (MDS) include a group of clonal myeloid neoplasms characterized by cytopenias due to ineffective hematopoiesis, abnormal blood and marrow cell morphology, and a risk of clonal evolution and progression to acute myeloid leukemia (AML). Because outcomes for patients with MDS are heterogeneous, individual risk stratification using tools such as the revised International Prognostic Scoring System (IPSS-R) is important in managing patients—including selecting candidates for allogeneic hematopoietic stem cell transplantation (ASCT), the only potentially curative therapy for MDS. The IPSS-R can be supplemented by molecular genetic testing, since certain gene mutations such as TP53 influence risk independent of established clinicopathological variables. For lower risk patients with symptomatic anemia, treatment with erythropoiesis-stimulating agents (ESAs) or lenalidomide (especially for those with deletion of chromosome 5q) can ameliorate symptoms. Some lower risk patients may be candidates for immunosuppressive therapy, thrombopoiesis-stimulating agents, or a DNA hypomethylating agent (HMA; azacitidine or decitabine). Among higher risk patients, transplant candidates should undergo ASCT as soon as possible, with HMAs useful as a bridge to transplant. Non-transplant candidates should initiate HMA therapy and continue if tolerated until disease progression. Supportive care with transfusions and antimicrobial drugs as needed remains important in all groups.

Enlarged spleen is associated with low neutrophil and platelet engraftment rates and poor survival after allogeneic stem cell transplantation in patients with acute myeloid leukemia and myelodysplastic syndrome

Article

Full-text available

Jun 2018
ANN HEMATOL

Primary graft failure can be a cause of early morbidity and mortality after allogeneic hematopoietic stem cell transplantation (HSCT), as it leads to a high risk of severe infections and bleeding. Splenomegaly is associated with primary graft failure in patients of myelofibrosis, but the association between splenomegaly and outcomes after HSCT in patients with myeloid malignancies has not been previously evaluated. The aim of this study was to investigate the effect of spleen volume on engraftment kinetics in patients with acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS). We enrolled 85 patients. The median spleen volume was 146 cm³ (quartile 88–201 cm³). The adjusted hazard ratios for neutrophil and platelet engraftments were 0.17 (0.07–0.40, p < 0.001) and 0.19 (0.05–0.69, p = 0.011), respectively, for the high-risk group, at a cutoff splenic volume of 320 cm³. Overall survival at 3 years after HSCT was significantly poor in the high-risk group with an adjusted hazard ratio of 13.8 (2.61–72.4, p = 0.002). Enlarged spleen was associated with low neutrophil and platelet engraftment rates and poor survival after allogeneic HSCT in patients of AML and MDS.

Benefits and risks of Splenectomy.

Article

Full-text available

Dec 2013
Int J Surg

Elroy Patrick Weledji

Splenectomy is a powerful therapeutic procedure in a wide variety of medical disorders provided that it is not undertaken lightly and the risks are weighed against the potential benefits in each individual case. Most of this risk seems to be due to the underlying splenectomy indication and not to splenectomy alone. There has been an increased tendency in recent years towards splenic preservation to prevent not only the risk of subsequent overwhelming post-splenectomy infection (OPSI) but the long term risk of cardiovascular complications. As there is no condition that can be cured by splenectomy, this paper reviewed the rationale behind the indications for, and the associated risks. Electronic searches of the medline (PubMed) database, Cochrane library, and science citation index were performed to identify original published studies on splenectomy. Relevant articles were searched from relevant chapters in specialized texts and all included.

Splenectomy In Patients with MDS

Article

Nov 2010

1879 Splenectomy in patients with MDS is a treatment option that is beeing applied very rare [Steensma D., et al, Leuk Res.,2003; Bourgeosis E., et al, Leukemia, 2001]. There are anecdotal reports with very few patients demonstrating its efficacy. In most cases splenectomy was indicated for MDS patients with immune related thrombocytopenia. Here we would like to report the results of 33 splenectomies in patients with MDS who have been treated in our Center during 1994–2010. Within this period of follow-up totally 155 patients were diagnosed with different forms of MDS, 35% of them presenting with hypoplasia. The MDS treatment algorithm in our Center incorporates splenectomy as one of the options for pts with hypoplastic forms of MDS with bone marrow blast count less than 10%, refractory to initial cyclosporin A treatment or refractory to transfusions. Among patients who were splenectomised there were 20 females, 13 males with a median age of 40 years (range 18–74). Median time from diagnosis to splenectomy was 12 months (range 4–107). By WHO-classification there were 2 patients with RA, 22 – with RCMD, 2 – with MDS and del (5q), 6 - with RAEB, 1 - with AML after MDS. Cytogenetic analysis was available in 32 cases, and karyotype was normal in 15 patients (47%).The most common abnormalities were: del (5q) - 3, del (20q) - 2, trisomy 8 - 2, tetrasomy 8 - 1, monosomy 7 - 2, complex karyotype - 4. Bone marrow biopsy revealed hypoplasia in 25 patients (75%), myelofibrosis – in 7 (21%). The median WBC count was 2,6*109/L (range 0,6-8,7), hemoglobin 6,9 g/dL (47-119) and platelets 26*109/L (6-170). 27 pts (82%) were RBC transfusion dependent, 22 (67%) - platelets transfusion dependent. 13 pts had received immunosuppression therapy (ATG, cyclosporine A) before splenectomy, 2 - cytotoxic chemotherapy, 3 - decitabine. The majority of splenectomies were done by laparoscopic method - 26 (79%), in one case the convertion was done. In all cases we performed liver biopsy. Postoperative complications (hemorrhage) occurred in 1 patient but there were no deaths due to operation. One death occurred in 7 days after splenectomy due to fulminant progression to AML. Median spleen weight was 180 gms (range 70–930). Median intraoperative blood loss was 250 ml (range 50–9350). Histology was available in 30 patients. Extramedullary hematopoesis was revealed in 3 cases (10%), blast infiltration - in 2 (7%), massive lymphoid infiltration was detected in 5 cases and in one patient in was proved to be clonal (marginal zone lymphoma, MZL). Hemosiderin depositions in the macrophages were seen in half of the cases -16 (53%). One case was characterized by granulomatosis in spleen and liver with negative immunohistohemical staining to Mycobacteria tuberculosis. Splenectomy lead to sustained improvement of cytopenias in 16 cases (48%): decreased transfusion dependence in 14 (42%) and transfusion independence in 2 (6%). After splenectomy 5 patients were followed by “wait and see” approach, 17 continued with immunosuppressive therapy (ATG,CyA), 3 patients were treated with cytotoxic chemotherapy, 1 – with decytabine, 2 received EPO, 1- danazol, 2 - iron chelation therapy, 2 – only transfusions therapy. We did not noticed the infections rate augmentation after splenectomy. Transformation to AML was registered 6 (18%) at median 6 months (0,3 -9). 13 splenectomized patients (39%) died at a median 12 months (range 0,3-84) and the main death reasons were: AML progression, aplasia deterioration followed by infections and hemorrhage. 20 patients are alive with a median follow-up after splenectomy 33 months (2-108). Analysis of our 15-years study data give us a confidence to conclude that splenectomy still may be an adequate option for distinct forms of MDS (hypoplastic forms with bone marrow blast count less than 10%, refractory to initial immunosupressive treatment or refractory to transfusions), producing cytopenia improvement in half of the patients with decreasing transfusion dependance also in half of the patients, sometimes bringing a clear diagnosis (MZL). The mechanism of action is not very clear but we can speculate that splenectomy removes the “cell-destroying” organ, deminishes immune pathways of cytopenias due to large lymphoid compartment deletion, provides the resustainment of sensitivity to immunosupressive agents. Disclosures No relevant conflicts of interest to declare.

Splenic infarction secondary to myelodysplastic syndrome: unravelling more etiologies

Article

Aug 2019

Myelodysplastic syndrome (MDS) is a neoplastic disorder resulting in dysplasia and apoptosis of the hematopoietic clonal cells. The presenting features of MDS are usually dependent on the cellular lineage affected in the bone marrow (BM). Generally, MDS presents in older adults with recurrent infections, anemia, and bleeding tendencies. However, until now, there are no cases of splenic infarction in MDS. Splenic infarction is a rare event and is often reported in myeloproliferative or thromboembolic disorders. In this case report, we present splenic infarction; a never reported clinical manifestation in an MDS patient.

Indications for and efficacy of splenectomy for haematological disorders

Article

May 2013
BRIT J SURG

Splenectomy is performed for a variety of indications in haematological disorders. This study was undertaken to analyse outcomes, and morbidity and mortality rates associated with this procedure. Patients undergoing splenectomy for the treatment or diagnosis of haematological disease were included. Indications for operation, preoperative risk, intraoperative variables and short-term outcomes were evaluated. From January 1997 to December 2010, 381 patients underwent splenectomy for diagnosis or treatment of haematological disease. Some 288 operations were performed by an open approach, 83 laparoscopically, and there were ten conversions. Overall 136 patients (35·7 per cent) experienced complications. Postoperative morbidity was predicted by age more than 65 years (odds ratio (OR) 1·63, 95 per cent confidence interval 1·05 to 2·55), a Karnofsky performance status (KPS) score lower than 60 (OR 2·74, 1·35 to 5·57) and a haemoglobin level of 9 g/dl or less (OR 1·74, 1·09 to 2·77). Twenty-four patients (6·3 per cent) died within 30 days of surgery. Postoperative mortality was predicted by a KPS score lower than 60 (OR 16·20, 6·10 to 42·92) and a platelet count of 50 000/µl or less (OR 3·34, 1·25 to 8·86). The objective of the operation was achieved in 309 patients (81·1 per cent). The success rate varied for each indication: diagnosis (106 of 110 patients, 96·4 per cent), thrombocytopenia (76 of 115, 66·1 per cent), anaemia (10 of 16, 63 per cent), to allow further treatment (46 of 59, 78 per cent) and primary treatment (16 of 18, 89 per cent). Splenectomy is an effective procedure in the diagnosis and treatment of haematological disease in selected patients.

Role of splenectomy in the treatment of myelodysplastic syndromes with peripheral thrombocytopenia: A report on six cases

Article

Jun 2001

Thrombocytopenia is generally of central origin in MDS, but can be due to peripheral platelet destruction in some cases. We studied platelet lifespan in 61 MDS cases with platelets < 70,000/mm3 and marrow blasts < 10%. Nine of them (15%) had a major platelet lifespan reduction (< 3.5 days), and were considered for splenectomy. Three of them were not splenectomized due to rapid death, patient refusal and older age plus liver predominance of platelet sequestration, respectively. The remaining six patients (two females and four males, median age 50 years, range 32 to 65) were splenectomized 3 to 21 months after diagnosis. Before splenectomy, five of them had RA and one had CMML. Platelets counts ranged from 5000 to 30,000/mm3 and did not durably respond to other treatments. Three of the patients has a relapse of platelet counts, concomitantly required platelet transfusion due to recurrent blending, whereas three had anemia (two required erythrocyte transfusion) and four had neutropenia. Three months after surgery, platelet counts ranged from 55,000 to 160,000/mm3 (> 100,000/mm3 in four cases), no patient required platelet or erythrocyte transfusion, but there was no effect on neutrophil counts. Three patients had a relapse of platelet counts, concomitant with progression to AML in two of them, whereas the third relapsing case achieved normal platelet counts with further danazol. One patient died with normal platelet counts 12 months after splenectomy (from sepsis, probably related to neutropenia rather than splenectomy). Two patients remained with normal platelet counts 10 and 52 months after surgery. Our findings suggest that the mechanism of thrombocytopenia should be studied more often in 'low risk' MDS (i.e. with low bone marrow blast counts) with thrombocytopenia, as about 15% of them appear to have peripheral platelet destruction. Some of those patients may benefit from splenectomy.

Splenic histology and histopathology: An update

Article

Jun 2003
SEMIN DIAGN PATHOL

Madeleine D Kraus

The spleen can be a troublesome specimen for the surgical pathologist, not only because experience with the range of "normal" splenic histology is limited by its rarity but also because there is an often a frustrating discordance between the patient's clinical condition and the perceived findings. Patients with a dramatic clinical presentation that points to splenic pathology ("hypersplenism" or marked splenomegaly) not infrequently have no discernable or have barely perceptible histologic abnormalities of the spleen. Similarly, patients whose spleens contain histologic findings that seem to deviate significantly from the "norm" (histiocytic proliferations, vasoformative lesions, stromal hyperplasia) may have no clinically detectable hematologic complaints. For most pathologists, the frame of reference for normal splenic histomorphology derives largely from experience with autopsy spleens and spleens removed for trauma or immune thrombocytopenia. These are all settings in which pre-existing disease, the immune status of the patient, and therapy influence the findings and--in cases in which fixation has been delayed--even the ability to make the findings. This review presents practical aspects of splenic development and immunoarchitecture and relates this to the pathologist's approach in evaluating the abnormal spleen and assists in resolving such discordances. Benign conditions that contrast with the subjects of subsequent articles in this issue are emphasized.

Outcomes following splenectomy in patients with myeloid neoplasms

Mar 2015
J SURG ONCOL
389-395

K L Rialon
P J Speicher
E P Ceppa
Dkk

Rialon KL, Speicher PJ, Ceppa EP, dkk. Outcomes following splenectomy in patients with myeloid neoplasms. J Surg Oncol. 2015 Mar 15;111(4):389-95

Is splenectomy a contraindication for patients with myelodysplastic syndromes complicated by splenomegaly?

Jan 2006
ANN HEMATOL
198-199

R Uchida
S Nakano
K Namura
Dkk

Uchida R, Nakano S, Namura K, dkk. Is splenectomy a contraindication for patients with myelodysplastic syndromes complicated by splenomegaly?. Ann Hematol. 2006;85(3):198-199.