Pancytopenia ec Hyperspleenism

with Liver Cirrhosis ec Chronic

Hepatitis B

Viqtor Try Junianto – RSUD Mimika

SMF Ilmu Bedah dan Anestesi
Case
Identity
• Name : Mr. AS
• Age : 50 yo
• Sex : Male
• RM : 13 86 87
• Address : SP 3
• Date of entry hospital : 26/06/2019
• Out of hospital : 06/07/2019
• Chief complaint :
Abdominal discomfort in the umbilicus area to the inguinal dextra
and suprapubik area for one year.

• History of the disease :

Abdominal pain which is described as an abdominal discomfort
for one year
The pain move around the abdomen from upper right to upper
left and finally move to lower right
The patient often feel malaise even when he is not going out for
work
His wife tell that the patient often looks like pale
Nausea (-), Vomit (-), Fever (-), Anorexia (-), weight loss (-)
Defecate and urinate normal
• Past illness history :
Liver disease (+)
Dm type II (+)

• Vital sign
BP : 110/70
HR : 60
RR : 20
Temperature : 36.5
SPO2 : 96
Vas : 1-2
Physical examination
• General condition : tsr, cm
• Head and neck : ca +/+, si -/-
• Thorax : breathing sound vesikuler/vesikuler
heart sound normal, murmur (-), gallop (-)
• Abdomen :
Inspkesi : supel, mass(-), surgery marks (-), caput medusa (-)
Palpasi : hepar tdk teraba, spleen palpable s4-s5
Perkusi : timpani
Auskultasi : bowel sound normal
• Extremity : akral hangat, crt <2 dtk, petechiae (-).
• Laboratory (26/06/2019)
Hb : 10 g/dl
Rbc : 4.81 (10^6/uL)
Hct : 31.5%
Mcv : 65.5 fL
Mch : 20.8 pg
Wbc : 2.54 (10^3/uL)
Neutrofil : 52.4%
Monosit : 17.3%
Trombosit : 42 (10^3/uL)

Albumin : 3.8 g/dl

SGOT : 28 U/lt
SGPT : 10 U/lt
Ureum : 13.7 mg/dl
Creatinin : 0.71 mg/dl
PT : 14 detik
APTT : 27.6 detik
INR : 1.30
• Temporary assessment : Pancytopenia ec Hypersplenism

• Planning :
Elective Open splenectomy 01/07/2019
Consule dr. Ronald SpAn : check for pt, aptt, ct, bt, and prepared
6 bags prc

• Laboratory (30/06/2019)
CT = 6.00”
BT = 2.30”
Follow up

01/07/2019 :
Patient takes operation, 02/07/2019 :
during operation the
liver to be found Patient take cbc test 03/07/2019
cirrhosis hepatik, after Internist (dr. Cornelia,
operation patient move Patient move to surgery
SpPD) : patient take observation ward
to HCU and consule to HbsAg, anti HCV, sgot,
internist sgpt, albumin, pt, aptt, Internist :
bilirubin total and Dx : sirosis hati
bilirubin direk test dekompensata ec hep.
B kronik + hipersplenism
sekunder
continue

03/07/2019
Internist :
06/07/2019 :
Planning : Diet hepar
Aminoleban inf 500 ml/day The patient can go
home with :
Spironolacton 25 mg 1-0-0
Propanolol 10 mg 0-1/2-0 Cefadroxil 500 mg/12 hr
Refer to SpPD-KGEH for Mefenamic acid 500
hbv-DNA and hbeAg mg/8 hr
Curcuma tablet/8 hr
Laboratory (02/07/2019)

Hb : 11.6 g/dl^
Rbc : 5.49 (10^6/uL)^
Hct : 36.7%^
Mcv : 66.8 fL^
Mch : 21.1 pg^
Wbc : 6.69 (10^3/uL)^
Trombosit : 75 (10^3/uL)^
continue
Albumin : 3.4 g/dl
SGOT : 39 U/lt
SGPT : 15 U/lt
D. Bil : 0.35 mg/dl
T. Bil : 0.54 mg/dl
PT : 11 detik
APTT : 25.7 detik
INR : 1.02
HbsAg : (+)
Anti HCV : (-)
Discussion
• Hypersplenism refers to a group of syndromes that involve
splenomegaly and peripheral cytopenia of various causes
• It’s characterized by : (1) splenomegaly, (2) pancytopenia or a
reduction in the number of one or more types of blood cells, (3)
normal production or hyperplasia of the precursor cells in the
marrow, (4) decreased red blood cells survival and (5)
decreased platelet survival
• This term was first introduced by Chauffaud in 1907

Elmakki E, Hypersplenism: Review article. Journal of Biology, Agriculture and Healthcare, Vol
2, No.10, 2012.
Splenic Anatomy and Function
• Spleen is the largest lymphoid organ of the body
• It plays important role in Red blood cells sequestration and
immunity
• Store house of platelets
• Produces RBC and WBC in fetus during gestation period and
some times in adults

Yunfu LV, Wan Yee Lau, Yejuan Li. Hypersplenism: History and current status (Review).
Experimental and Therapeutic Medicine 12: 2377-2382, 2016.
• Located between 9th and 11th ribs on left side
• Red organ measuring 3×8×14 cm
• Weighing 100-157 gm
• Histologically there are two main anatomical components :
• The red pulp (lined by endothelial macrophages and cords)
• The white pulp (similar structure to lymphoid follicles)

Yunfu LV, Wan Yee Lau, Yejuan Li. Hypersplenism: History and current status (Review).
Experimental and Therapeutic Medicine 12: 2377-2382, 2016.
Blood Supply

Yunfu LV, Wan Yee Lau, Yejuan Li. Hypersplenism: History and current status (Review).
Experimental and Therapeutic Medicine 12: 2377-2382, 2016.
Yunfu LV, Wan Yee Lau, Yejuan Li. Hypersplenism: History and current status (Review).
Experimental and Therapeutic Medicine 12: 2377-2382, 2016.
Functions of spleen
• Clearance of microorganisms and particulate antigens from the
blood stream
• Synthesis of immunoglobulin G (IgG), properdin, and tuftsin
• Removal of abnormal red blood cells (RBCs)
• Extramedullary hematopoiesis in certain diseases

Yunfu LV, Wan Yee Lau, Yejuan Li. Hypersplenism: History and current status (Review).
Experimental and Therapeutic Medicine 12: 2377-2382, 2016.
Hyperspleenism
• Hypersplenism is a condition in which the spleen becomes
increasingly active and then rapidly removes the blood cells
• Size 2 to 3 times the size spleen is palpable
• Weight 400-500 gms
• Spleen size is not a reliable indicator of spleen function

Liang Li, Mubing Duan, Weisan Chen. The spleen in liver cirrhosis: revisiting an old enemy
with novel targets. Li et al. J Transl Med (2017) 15:111.
Clinical features
• The most common complaint in patients with splenomegaly is
mild, vague, abdominal discomfort
• Febrile illness
• Pallor, dyspnea, bruising, and/or petechiae
• History of liver disease
• Weight loss, constitutional symptoms
• Alcoholism, hepatitis
• Family history should be reviewed to disclose relevant
hereditary diseases

https://emedicine.medscape.com/article/206208
Physical Examination
• Mass in left hypochondrium
• Notch felt
• Moves with respiration
• Dull on percussion
• Directed toward Rt iliac fossa
• Hook sign

https://emedicine.medscape.com/article/206208
Liang Li, Mubing Duan, Weisan Chen. The spleen in liver cirrhosis: revisiting an old enemy with
novel targets. Li et al. J Transl Med (2017) 15:111.
Approach Considerations
• Complete blood cell count (CBC) with differential
• Liver function testing
• Hepatitis B and C testing
• Lactate dehydrogenase (LDH)
• Erythrocyte sediumentation rate (ESR)
• Evaluation of peripheral blood smear for RBC morphology and
signs of myeloproliferative disorders or underlying bone
marrow disorders
• Prothrombin time with international normalized ratio (INR) and
activated partial thromboplastin time (aPTT)

https://emedicine.medscape.com/article/206208
Continue
• Imaging studies (USG, CT-Scan, Angiographic findings,
Splenoportography)
• Liver-Spleen Colloid Scanning
• Biopsy
• Histologic Findings

https://emedicine.medscape.com/article/206208
Classification of hypersplenism
• Primary hypersplenism. Examples are primary splenic
hyperplasia, primary splenic granulocytopenia, primary splenic
pancytopenia
• Secondary hypersplenism. Examples include i) infections; ii)
alcohol use; iii) malignancies

Yunfu LV, Wan Yee Lau, Yejuan Li. Hypersplenism: History and current status (Review).
Experimental and Therapeutic Medicine 12: 2377-2382, 2016.
Etiology
• Infectious causes

• Hyperplastic splenomegaly : Hereditary spherocytosis,

thalasaemia, myelofibrosis, and Chronic myeloid leukaemia

• Congestive splenomegaly : Liver cirrhosis

• Infiltrative splenomegaly : Gaucher's disease, amyloidosis,

splenic tumours, and metastatic malignancy

• Micellanous causes

Elmakki E, Hypersplenism: Review article. Journal of Biology, Agriculture and Healthcare, Vol
2, No.10, 2012.
Causes of massive spleenomegaly
• visceral leishmaniasis (kala-azar)
• chronic myelogenous leukemia
• myelofibrosis
• malaria
• primary lymphoma of spleen
• J. Lemaire, A. Rosière, C. Bertrand. Surgery for massive
splenomegaly. BJS Open 2017; 1: 11–17.

J. Lemaire, A. Rosière, C. Bertrand. Surgery for massive splenomegaly. BJS Open 2017; 1:
11–17.
Liver and Spleen Crosstalk Pathways during Liver Cirrhosis

Liang Li, Mubing Duan, Weisan Chen. The spleen in liver cirrhosis: revisiting an old enemy with
novel targets. Li et al. J Transl Med (2017) 15:111.
Management of Hypersplenism
• Non-surgical treatment. This category of treatment primarily
includes etiological treatment and treatment of concomitant
diseases.
• Surgical treatment
• Splenectomy
• Splenic embolization

https://emedicine.medscape.com/article/206208
Treatment of Postsplenectomy Infection

• Fulminant, life-threatening infection represents a major long-

term sequela after splenectomy in patients with splenomegaly
• Overwhelming postsplenectomy infection (OPSI)
• Most instances of serious infection are due to encapsulated
bacteria
• Education, vaccination, and antibiotic prophylaxis

https://emedicine.medscape.com/article/206208
References
• Elmakki E, Hypersplenism: Review article. Journal of Biology,
Agriculture and Healthcare, Vol 2, No.10, 2012.
• Yunfu LV, Wan Yee Lau, Yejuan Li. Hypersplenism: History and
current status (Review). Experimental and Therapeutic
Medicine 12: 2377-2382, 2016.
• Liang Li, Mubing Duan, Weisan Chen. The spleen in liver
cirrhosis: revisiting an old enemy with novel targets. Li et al. J
Transl Med (2017) 15:111.
• J. Lemaire, A. Rosière, C. Bertrand. Surgery for massive
splenomegaly. BJS Open 2017; 1: 11–17.
• https://emedicine.medscape.com/article/206208