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A 37-Year-Old Female Patient with Recurrent Thrombocytopenia, Systemic Lupus

Erythematosus, Azotemia, History of Abortion Liver Cirrhosis with Dyslipidemia

M. Rosyid Ridho1, Rakhma Yanti Hellmi2, Damai Santosa2, Didik Indiarso3 , ,

1
Department of Internal Medicine, Faculty of Medicine, Diponegoro University, Dr.Kariadi
Central General Hospital, Semarang, Indonesia
2
Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Diponegoro
University, Dr.Kariadi Central General Hospital, Semarang, Indonesia
3
Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Diponegoro
University, Dr.Kariadi Central General Hospital, Semarang, Indonesia
3
Division of Gastroenterohepatology, Department of Internal Medicine, Faculty of Medicine,
Diponegoro University, Dr.Kariadi Central General Hospital, Semarang, Indonesia
Abstract

Background:
SLE (Systemic Lupus Erythematosus) is an autoimmune disease that manifests itself in a variety
of ways. Thrombocytopenia is a symptom of SLE that is frequently associated with
rash/petechiae, bruising, or bleeding in patients. 1 Hematological laboratory results and ANA
Profile results confirmed the patient's diagnosis of systemic lupus erythematosus (SLE). This
patient also had cirrhosis of the liver, which was suspected to be caused by non-alcoholic
steatohepatitis.

Case report:
A 37-year-old woman came to Dr. Kariadi Central General Hospital with a hematoma on her
lower extremity and back with bleeding gums.11 years ago the patient had a history of
miscarriage at 3 months of gestation. and until now the patient has never been pregnant again,
the patient has had no children with infertility Secunder. When the first admision history patient
had a history of recurrent thrombocytopenia, they got therapy inj Methylprednisolon 62,5 mg/12
hours and a blood transfusion of Trombcyt concentrate 4 kolf, TC apheresis 300 cc, but the
trombocyt level was not increased. Laboratory examination showed normochromic normocytic
anemia (Hb 10.6 g/dL) and thrombocytopenia (13,000/uL). PPT/PTTK 12.6/19.9 D-Dimmer
4010, Fibrinogen 402, Urea 56, Creatinine 1.5 eGFR 58 mL/min/1.73 m2, SGOT 27, SGPT 32,
Proteinuria + (50), HBsAg (–), Anti-HCV Negative (–), Direct Coombs Test (–), Indirect
Coombs Test (–),The Patient's Second admision History Laboratory Results: Hemoglobin 9,8,
Leukosit 7, Trombocyt 36, Total Cholesterol 208, Triglycerides 231, HDL 43, LDL 158.
Fibroscan examination Liver stiffness results: 14.8 kPa according to the Metavir F4 scale; ANA
Profile SSS-A results in native (++), Jo-1 (+++), and Ro-52 recombinant (+++); SLE
classification ACR 2019 got a score of 7, meet criteria for SLE with Thrombocytopenia and
nephritis. The patient also had liver cirrhosis, which was presumed to be caused by nonalcoholic
fatty liver disease. This disease is thought to cause recurrent thrombocytopenia. This patient was
treated with Inj Methylprednisolone 62.5 mg/12 hours, Eltrombopag 1x 50 mg, Cyclosporine 50
mg/8 hours, and Atorvastatin 20 mg/24 hours.

Discussion:
Systemic Lupus Erythematosus (Systemic Lupus Erythematosus or SLE) is an autoimmune
disease that has a wide variety of symptoms. 1 The typical clinical manifestations of SLE include
a red rash, a discoid rash on the face, the manifestation of bleeding starting from a petechial
rash, mucosal bleeding, or infrequent bleeding. Mucocutaneous bleeding appears in the form of
petechiae, purpura, and ecchymosis of the skin.3 Hematologic manifestations include iron
deficiency anemia, chronic disease anemia, autoimmune hemolytic disease, drug toxicity,
thrombocytopenia, leukopenia, lymphopenia, and elevated ESR.6 The results of the ANA Profile
examination were also positive, indicating that the patient had SLE, so immunosuppressant
therapy was the right choice. This patient also had dyslipidemia with liver cirrhosis through a
fibroscan examination. Liver cirrhosis is thought to be due to dyslipidemia or NAFLD
(Nonalcoholic Fatty Liver Disease).7 There are several histological stages in the scientific
course of NAFLD that describe the progression of the existing lesions. The stages include fatty
liver only, steatohepatitis (NASH), steatohepatitis with fibrosis, and ultimately cirrhosis.7

Conclusion:
Clinically, patients with recurrent thrombocytopenia should be monitored for the possibility of
incidental lupus. Early diagnosis, suspecting atypical symptoms in the patient, and early
treatment can prevent LES progression so that early remission can be achieved. In addition, a
patient with a history of recurrent thrombocytopenia who had transfusion therapy and inj steroid
therapy must think of other causes. like Non-Alcoholic Steatohepatitis. In this case, additional
examination is needed, to establish a definite diagnosis of liver cirrhosis, like liver biopsy.

Key words: ITP, Immune Thrombocytopenic Purpura, Thrombocytopenia, SLE, Systemic Lupus
Erythematosus, Cirrhosis, NAFLD Bibliography:
1. Stojan G, Petri M. Epidemiology of systemic lupus erythematosus: An update [Internet].
Vol. 30, Current Opinion in Rheumatology. Lippincott Williams and Wilkins; 2018 [dikutip
27 April 2021]. hal. 144–50. Tersedia pada: /pmc/articles/PMC6026543/
2. Kistangari G, McCrae KR. Immune thrombocytopenia. Hematol Oncol Clin North Am.
2013;27(3):495-520. doi:10.1016/j.hoc.2013.03.001
3. Aringer, M., Costenbader, K., Daikh, D., Brinks, et.al. (2019). 2019 European League
Against Rheumatism/American College of Rheumatology Classification Criteria for
Systemic Lupus Erythematosus. Arthritis & rheumatology (Hoboken, N.J.), 71(9), 1400–
1412. https://doi.org/10.1002/art.40930
3. Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Provan D, et. al. Standardization of
terminology, definitions and outcome criteria in immune thrombocytopenic purpura of
adults and children: report from an international working group. Blood. 2009 Mar
12;113(11):2386-93.
4. Neunert C, Noroozi N, Norman G, Buchanan GR, Goy J, Nazi I, Kelton JG, Arnold DM.
Severe bleeding events in adults and children with primary immune thrombocytopenia: a
systematic review. J Thromb Haemost. 2015 Mar;13(3):457-64.
5. Beisel C, Weiler-Normann C, Teufel A, Lohse AW. Association of autoimmune hepatitis
and systemic lupus erythematodes: a case series and review of the literature. World J
Gastroenterol. 2014;20(35):12662-12667. doi:10.3748/wjg.v20.i35.12662
6. Perhimpunan Reumatologi Indonesia. Diagnosis dan Pengelolaan Lupus Eritematosus
Sistemik. In: Sumariyono, Kalim H, Setyohadi B, Hidayat R, Hamijoyo L, Ariane A, editor.
Jakarta: Perhimpunan Reumatologi Indonesia; 2019. hal. 130
7. Li B, Zhang C, Zhan YT. Nonalcoholic Fatty Liver Disease Cirrhosis: A Review of Its
Epidemiology, Risk Factors, Clinical Presentation, Diagnosis, Management, and Prognosis.
Can J Gastroenterol Hepatol. 2018;2018:2784537. Published 2018 Jul 2.
doi:10.1155/2018/2784537
8. You H, Peng L, Zhao J, et al. Clinical Characteristics of Systemic Lupus Erythematosus
with Cirrhosis. J Immunol Res. 2020;2020:2156762. Published 2020 May 21.
doi:10.1155/2020/2156762
Antigen          
RNP /Sm (RNP/Sm)          
Sm (Sm)          
SS-A native (60 kDa) (SSA) ++        
Ro- 52 recombinant (52) +++        
SS-B (SSB)Scl-70(Scl)          
PM-Scl100 (PM100)          
Jo-1 (Jo) +++        
Centromere B (CB)          
PCNA (PCNA)          
dsDNA(DNA)          
Nucleosomes (NUC)          
Histones (H)          
Ribosomal Protein (RIB)          
AMA-M2 (M2)          
DFS70 (DFS70)          
Control (Co) +++        

  13/2/22 16/2/22 22/2/22 18/2/22 14/4/22 1/2/22 7/4/22

Hemoglobin 7.4 7.2 10.1 9.2 9.3 2.9 9.8

Hematokrit 23.7 23 32 28.7 29.8 9.8 31.2

MCH 25.6 25.4 26.6 26 31.5 26.9 31

MCV 82 81.3 84.4 81.1 101 90.7 98.7

MCHC 31.2 31.3 31.6 32.1 31.2 29.6 31.4

Leukosit 7.7 11 5.6 7.1 10.3 25.9 7

Trombosit 3 9 136 23 18 8 36

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