Splenomegaly is a condition which cannot be treated but can be cured by reducing the symptoms. if untreated can lead to life-threatening conditions.

1. Case presentation on splenomegaly with portal
hypertension
J. Anisha Ebens
Pharm D intern

2. Splenomegaly:
• Splenomegaly is defined as enlargement of the
spleen, measured by size or weight.
• The spleen is a functionally diverse organ with
active roles in immunosurveillance and
hematopoiesis. It lies within the left upper quadrant
of the peritoneal cavity.
• A normal spleen weighs 150 g and is
approximately 11 cm in craniocaudal length.
• A spleen weight of 400-500 g indicates
splenomegaly, while a weight of more than 1000 g
is labelled massive splenomegaly. Poulin et al
defined splenomegaly as moderate if the largest
dimension is 11-20 cm, and severe if the largest
dimension is greater than 20 cm.

3. In many instances, the spleen enlarges as it performs its normal
functions. The four most important normal functions of the spleen are
as follows:
• Clearance of microorganisms and particulate antigens from the blood
stream
• Synthesis of immunoglobulin G (IgG), properdin (an essential
component of the alternate pathway of complement activation), and
tuftsin (an immunostimulatory tetrapeptide)
• Removal of abnormal red blood cells (RBCs)
• Extramedullary hematopoiesis in certain diseases
Causes:
• Although a wide variety of diseases are associated with enlargement
of the spleen, the following six etiologies of splenomegaly are
considered primary:
• Immune response work hypertrophy - Such as in subacute bacterial
endocarditis or infectious mononucleosis
• RBC destruction work hypertrophy - Such as in hereditary
spherocytosis or thalassemia major
• Congestive - Such as in splenic vein thrombosis, portal hypertension,
or Banti disease
• Myeloproliferative - Such as in chronic myeloid metaplasia

4. Miscellaneous causes of splenomegaly include the following:
• Trauma
• Cysts
• Hemangiomas
• Metastasis
• Giant abscess (see the images below)
• Certain drugs (eg, Rho(D) immune globulin)
Types:
• Inflammatory splenomegaly
• Hyperplastic splenomegaly
• Congestive splenomegaly
• Infiltrative splenomegaly
• Infectious splenomegaly
• Splenic sequestration

5. Epidemiology:
Race-, Sex-, and Age-related demographics
• No race predilection is recognized for splenomegaly. However, note
that blacks may have hemoglobin SC disease, a disorder related to
sickle cell disease. Unlike sickle cell disease, which results in a
small, autoinfarcted spleen, patients with hemoglobin SC disease
may have splenomegaly that accompanies their pigment gallstones.
• Tropical splenomegaly syndrome (or hyperactive malarial syndrome)
has a female-to-male incidence ratio of 2:1. Otherwise, no sex
predilection is documented for splenomegaly.
• The capsules of older spleens are much thinner than their younger
counterparts. The combination of capsular thinning with increased
spleen weight and size makes splenic injury more common in elderly
persons. These factors account for the increased likelihood
of splenectomy for trauma in this subgroup.

6. Symptoms:
The most common complaint in patients with splenomegaly is
mild, vague, abdominal discomfort. Patients may also experience
pain,which may be referred to the left shoulder. Increased
abdominal girth is less common. Early satiety from gastric
displacement occurs with massive splenomegaly. Associated
symptoms or signs are typically related to the underlying
disorder and may include the following:
• Febrile illness (infectious)
• Pallor, dyspnea, bruising, and/or petechiae (hemolytic process)
• History of liver disease (congestive)
• Weight loss, constitutional symptoms (neoplastic)
• Pancreatitis (splenic vein thrombosis) [8]
• Alcoholism, hepatitis (cirrhosis)
Additional signs that identify possible etiologies of splenomegaly
include the following:
• Signs of cirrhosis - Eg, asterixis, jaundice, telangiectasias,
gynecomastia, caput medusa, and ascites
• Heart murmur - Endocarditis or congestive failure
• Jaundice
• Scleral icterus - Spherocytosis or cirrhosis
• Petechiae - Any other bleeding manifestation secondary to
thrombocytopenia

7. Diagnosis:
Physical Examination
• Splenic size is not a reliable guide to splenic function, and palpable
spleens are not always abnormal. Patients with chronic obstructive
pulmonary disease (COPD) and low diaphragms commonly have
palpable spleens.
• The physical examination should include palpation with the patient in
the supine and right lateral decubitus position, with knees up and
hips flexed. Apply light fingertip pressure as the patient slowly
inspires. The use of the reverse Trendelenburg position may aid in
bringing the spleen into contact with the examiner's fingers. This is
especially helpful in patients with morbid obesity. The spleen moves
with respiratory patterns and may be palpable only at the end of
inspiration.
Conditions to consider in the differential diagnosis of massive
splenomegaly include the following:
• Leishmaniasis
• Malaria
• Myeloproliferative disease
• Portal vein obstruction/portal hypertension
• Schistosomiasis
• Mucopolysaccharid
osis
• Lymphomas
• Gaucher disease
• Hereditary
spherocytosis
• Thalassemias –
major, alpha or
beta
• Histiocytosis X

8. Conditions to consider in the differential diagnosis of mild to moderate
splenomegaly include all of the above, as well as the following:
• Hyperlipidemias
• Idiopathic splenomegaly
• Immune hemolytic anemias
• Immune thrombocytopenic
disorders
• Leukocyte disorders
• Ovalocytosis
• Splenic vein obstruction
• Symptomatic human
immunodeficiency virus
(HIV) infection
• Trypanosomiasis
• Congestive heart failure
• Drug reactions with serum
sickness syndromes
• Bacterial sepsis
• Infective endocarditis
• Sickle cell anemia
• Splenic abscess
• Acute infectious illnesses (eg,
typhoid, malaria,
• other tropical diseases)
• Acute viral infections (eg,
infectous mononucleosis)
• Systemic lupus
erythematosus
• Tuberculosis
• Angioimmunoblastic
lymphadenopathy
• Banti disease

9. On laboratory testing, if the sedimentation rate is high, the
differential diagnosis includes the following:
• Infection
• Acute leukemias
• Sarcoidosis
• Other inflammatory disorders
If the sedimentation rate is low, the differential diagnosis
includes the following:
• Chronic myeloproliferative disorders
• Hereditary hemolytic anemias
• Infiltrative disorders
Clinically detected splenomegaly is confirmed and quantified
using imaging studies. Ultrasonography is a noninvasive, highly
sensitive, and specific imaging technique for the evaluation of
splenic size.
Angiographic findings are used to differentiate splenic cysts from
other splenic tumors. Splenoportography is used to evaluate
portal vein patency and the distribution of collateral vessels
before shunt operations for cirrhosis. Splenoportographic findings
can help to identify the cause of idiopathic splenomegaly,
especially in children.
A CT scan remains the most useful preoperative investigation to
measure splenic volume

10. Treatment:
Treatment for an enlarged spleen focuses on the
underlying problem. For example, if you have a bacterial
infection, treatment will include antibiotics.
• Watchful waiting
• Spleen removal surgery
Reducing infection risk after surgery
• After spleen removal, certain steps can help reduce
your risk of infection, including:
• A series of vaccinations both before and after the
splenectomy. These include the pneumococcal
(Pneumovax 23), meningococcal and haemophilus
influenzae type b (Hib) vaccines, which protect against
pneumonia, meningitis and infections of the blood,
bones and joints. You'll also need the pneumococcal
vaccine every five years after surgery.
• Taking penicillin or other antibiotics after your operation
and anytime you or your doctor suspects the possibility
of an infection.
• Calling your doctor at the first sign of a fever, because
this may indicate an infection.

11. Case study on splenomegaly with portal hypertension

12. A female patient of age 55, was admitted in GM on
15.08.2018
C/O: Abd. Pain R hypochondrium x 1 month
R side Shoulder pain +
R chest pain +
Nausea +.
H/O: R side chest pain + daily, dull, aching in nature.
No H/O trauma/ injury
Past Medical History:
K/C/O- DM x 3yrs on Rx
HTN x 1yr on Rx
Past Medication History: T. Metformin 500mg BD
T. Januvia 25mg OD
T. Amlong 2.5mg BD
T. Propranolol 40mg BD

13. Surgical history: Pregnancy – caesarean - then operated
again for threat formation.
2016 – Esophagus band - for hole in it (C/O blood
vomiting)
Esophagus – Varices Grade I & II column, Mild PHG
Stomach – Mosaic pattern
Personal History: Takes mixed diet, Attained menopause 4
yrs back
Family History: Mother had SHT in old age
Vitals: Normal, patients was conscious and oriented
General Examination:
Temp.: Afebrile BP:
PR : 80 beats/min RR: 20 breaths/min
16/8 17/8 19/8
140/90 130/80 130/80

14. Systems examination:
CVS: S₁S₂ + RS: NVBS +
CNS: NFND P/A: soft
Lab investigation:
Parameters Report values Normal values
HB 9.5 12 – 16 g/dl
TLC 10100 3800 – 11000 Cells/mm³
DC N-69, L-26, E-5 N: 45-75, L: 16-46, E: 0-8
RBS 189 70- 160 mg/gL
FBS 112 70 – 110 mg/dL
PPBS 185 <140 mg/dL
BUN 20 8 – 25mg/dL
Cr 0.6 0.5 – 1.1 mg/dL
T. Chol (Previous test - 178) 229 <200mg/dL
Liver
Profile
Normal study

15. Other investigation:
CECT Abd, ECG
Impression: ECG: Moderately Left Ventricular
Hypertrophy, Bradycardia
CECT: Spleenomegaly – 13.1 cm, Mild dilated
and tortuous splenic vein. Mild dilated portal vein &
Splenic vein (13.9 mm). Diffuse mild bladder wall
prominence – to rule out cystitis
Diagnosis: Spleenomegaly with portal hypertension,
Past case of esophagal varices grade II with
severe PHG
T2DM and SHT.

16. Day 1:
C/O Abd. Pain, shoulder pain, chest pain, nausea. Her
vitals were normal. Blood sample was sent for CBC report.
Inj. Rantac, Inj. Emeset, T. BCT, T. Cal, T. MF,
T. Januvia, T. Atorvas, T. Amlong, T. Propranolol was
given on admission.
Day 2:
C/O Abd. Pain +. Vitals were normal.
Same drug therapy continued with addition of Inj.
Dicyclomine.
Day 3:
Abd. Pain reduced.
Same drugs continued with addition of combiflam gel.

17. Day 4:
C/O rigors, Abd. Pain reduced.
T. Cetirizine + Montelukast, T. Para was added along with
the previous drugs.
Day 5:
C/O rigors subsided, Her symptoms improved.
Inj. Dicyclomine and T. Calcium was stopped.
Day 6:
Inj. Emeset was stopped. And the patient was discharged.

18. Drug Chart:
S.N
o
Drug name Dose ROA Freq. No. of days
1 Inj. Ranitidine 50mg IV BD 15 - 20
2 Inj. Ondansetron 4mg IV BD 15- 19
3 Inj. Dicyclomine 10mg IM OD 16 - 18
4 T. BCT 1 tab P/O OD 15 - 20
5 T. Calcium 500mg P/O OD 15 - 19
6 T. MF 500mg P/O TDS 15 - 20
7 T. Sitagliptin 25mg P/O OD 15 - 20
8 T. Atorvastatin 10mg P/O 2HS 15 - 20
9 T. Amlodipine 2.5mg P/O BD 15 - 20
10 T. Propranolol 40mg P/O ½ BD 15 - 20
11 Combiflam gel 1 pinch E/A SOS 17 - 20
12 T. Para 500mg P/O TDS 18 - 20
13 T. Montek LC 4mg P/O 1-0-1 18

19. Discarge advice:
Patient discharged on 20.08.2018 with the following drugs
T. Rantac 150mg BD
T. BCT 1tab OD
T. Metformin 500mg BD
T. Sitagliptin 25mg BD
T. Atorvas 10mg 2HS
T. Propranolol 40mg BD
T. Para 500mg SOS
The patient was asked to review after 1 week.

20. FARM Notes:
Findings: Major interaction: Calcium & Antihypertensives
Amlodipine & Metformin
Assesment: Calcium decreases the effect of
Antihypertensives.
Amlong decrease the effect of Metformin
Resolution: Calcium can be stoopped. Concurrent
administration of Metformin and Amlong can be stopped.
Monitoring: Monitor closely.

21. Patient Counselling:
• These patients must be cautioned about contact sports and other
activities that may acutely increase intra-abdominal pressure or
place excessive forces on the left upper quadrant, left flank, or
lateral back. This decreases the likelihood of splenic rupture in a
patient with an abnormal splenic mass and capsule. The routine use
of seat belts is essential while driving or riding in a motor vehicle.
• Asplenic patients should be encouraged to wear a medical alert
bracelet and carry a wallet card explaining their lack of a spleen.
Patients should also be aware of the need to notify their physician in
the event of an acute febrile illness, especially if it is associated with
rigors or systemic symptoms.