Keywords

Massive splenomegaly, portal hypertension, hepatic cirrhosis

Introduction

Massive splenomegaly is defined as weight of spleen >1,000 g or largest dimension >20 cm. It is usually reported in myeloproliferative disorders, lymphoma, leukemia, visceral leishmaniasis, tropical malaria and extrahepatic (noncirrhotic) portal hypertension. We describe herein one unusual case of massive splenomegaly in a patient of hepatic cirrhosis with portal hypertension.

About the Author

Associate ProfessorSenior Resident1st Year ResidentJLN Medical College, Ajmer, RajasthanAddress for correspondenceDr Monika MaheshwariNaveen Niwas, 434/10, Bapu Nagar, Ajmer, Rajasthan

Case Report

A 45-year-old female, diagnosed to have chronic liver disease 3 years back, presented with fever anddragging sensation in the abdomen along with abdominal distension and difficulty in breathing since 15 days. On physical examination, she looked pale, mild icteric and toxic. Abdomen was nontender with massive splenomegaly and moderate ascites. There was reduced air entry in left hemithorax. Laboratory investigation revealed hemoglobin (Hb) - 8 g/dL, total leukocyte count (TLC) - 2,100/mm3, neutrophils - 59%; lymphocytes - 28% and platelets at 38,000/mm3. Stool was positive for occult blood. Bilirubin was 2.5 mg/dL, with direct bilirubin at 0.46 mg/dL, alanine aminotransferase (ALT) at 94 U/L, alkaline phosphatase (ALP) at 112 U/L and albumin at 2.5 g/dL.

This case was investigated for other causes of massive splenomegaly including tropical splenomegaly, malaria, noncirrhotic portal hypertension and leishmaniasis. Her anti-HCV (hepatitis C virus), anti-HDV (hepatitis D virus), antinuclear antibodies, malarial parasite and Coomb’s test was negative. Bone marrow aspirate and trephine revealed hypercellular marrow with all cell lines normal. Serum ceruloplasmin and iron studies were normal. Ultrasound of the abdomen demonstrated massive splenomegaly of size 24 cm, increased portal vein diameter (>20 mm), contracted gallbladder and liver and left pleural effusion with gross ascites. Computed tomography (CT) scan of abdomen also confirmed massive splenomegaly with dilated splenic and superior mesenteric vein shrunken liver with irregular borders and porcelain gallbladder with cholelithiasis (Figs. 1 and 2). Patient was referred to higher center for hybrid management of massive splenomegaly and pancytopenia.

Discussion

Splenomegaly is a common finding in a wide-spectrum of diseases. In a retrospective study, evaluating massive splenomegaly in 2,056 patients who presented to a large university medical center from 1913 to 1995, 31% had a hematologic disorder (lymphoma, leukemia), 17% had hepatic disease (noncirrhotic portal hypertension) and 8% had infectious disease (visceral leishmaniasis, tropical malaria).1 Massive splenomegaly is a rarely described feature of hepatic cirrhosis.2

In this case report, we shared our experience with recently encountered one case of hepatic cirrhosis who presented with massive splenomegaly crossing the midline. Massive splenomegaly caused mechanical problem of heaviness/distension on the left side of the abdomen along with pancytopenia. The exact relationship between the enlarged spleen and the decrease in cellular elements of the blood is unknown but two interesting theories have been proposed. Doan3 suggests that with stagnation of the blood in the spleen, there is a change in the cells which makes them more susceptible to hemolysis. Dameshek4 on the other hand, believes that the spleen elaborates a hormone, which acts on the marrow to suppress it so that the cells do not mature and hence do not reach the general circulation. Although systemic therapies are usually the primary modality of treatment for most disorders causing massive splenomegaly, splenectomy may be helpful. Studies have shown that laparoscopic splenectomy can sometimes be performed even for massive spleens, with a resultant decrease in blood loss, a shorter postoperative hospital stay and a decrease in postoperative morbidity and mortality.5 However, overwhelming postsplenectomy septicemia risk should be reduced with the administration of preoperative polysaccharide vaccines against Streptococcus pneumoniae, Haemophilus influenzae and Neisseria meningitides.

Conclusion

This case report highlights that cirrhotic portal hypertension may be added in the differential diagnosis of massive splenomegaly to facilitate timely diagnosis and treatment as we did in our patient.

References

1. O’Reilly RA. Splenomegaly in 2,505 patients at a large university medical center from 1913 to 1995. 1963 to 1995: 449 patients. West J Med 1998;169(2):88-97.
2. Mirza R, Z A, Z A, Nh L. Massive splenomegaly with pancytopenia in children and adolescents with hepatitis B cirrhosis. J Coll Physicians Surg Pak 2006;16(9):629-30.
3. Doan CA. Ibid 1949;25:625-7
4. Dameshek W. Hypersplenism. Bull N Y Acad Med 1955;31(2):113-36.
5. Owera A, Hamade AM, Bani Hani OI, Ammori BJ. Laparoscopic versus open splenectomy for massive splenomegaly: a comparative study. J Laparoendosc Adv Surg Tech A 2006;16(3):241-6.