Splenomegaly is characterized by enlargement of the spleen, distinct from hypersplenism, denoting an excessively active spleen. This condition constitutes a nonspecific clinical manifestation frequently associated with a variety of underlying etiologies, including infectious diseases such as malaria, leishmania, and schistosomiasis, hematological disorders such as sickle cell disease and hemoglobinopathy, infiltration of the spleen by malignant neoplasms, portal hypertension, immunological dysfunction, liver cirrhosis, metabolic disorders including amyloidosis and in Gaucher disease, and extramedullary hematopoiesis[1].During pregnancy,the appearance of massive splenomegaly is a relatively rare condition,and its impact can be exacerbated by concurrent complications such as anemia,thrombocytopenia, ascites,and jaundice[2].

Despite of little data, which is available in the literature concerning the obstetric outcomes in patients with splenomegaly, there is an evidence of significant association of massive splenomegaly, anemia, fever and chronic abdominal pain during pregnancy [3] Splenomegaly might be a predictor for poor obstetric outcome in particular intrauterine growth restriction.

A 24-year-old primigravida presented at 20 weeks of gestation with pancytopenia attributed to significant splenomegaly,with the spleen measuring approximately 14x7cm on examination. Ultrasound at 20 weeks showed an enlarged spleen of 18 cm, which further enlarged to 21 cm by 34 weeks.

Fig 1: Ultrasonographic image of spleen measuring 19.64cms

Fig 2,3: Altered liver texture with HEMANGIOMA in right lobe of liver and dilated portal vein-CLD with PORTAL HYPERTENSION

Laboratory findings revealed deranged liver function tests suggestive of a myeloproliferative disorder. Complete blood counts performed biweekly, consistently showed anemia with thrombocytopenia with platelet counts as low as 40,000/µL. Patient received packed red blood cells and multiple single donor platelet transfusions.

She was started on Colony-stimulating factors (Inj.Filgastrin 300 mg s/c od for 5 days), Thrombopoietin receptor agonists (T.Elthrombopag 50mg od )  and Erythropoiesis-stimulating agents (Inj.Recombinant Erythropoietin 2000 units s/c)  along with Steroids (T.Prednisolone 30mg od). Vaccinations against Pneumococcus, Influenza, and Meningococcus were administered during the antenatal period. Anti-D immunoglobulin was given thrice (600 mcg IM each) in  third trimester.

A multidisciplinary team comprising Internal medicine, Obstetrician, Gastroenterology, and Hematooncology was involved.Upper gastrointestinal endoscopy revealed severe gastritis with ?Antral polyp, leading to the initiation of proton pump inhibitors.

Fig 4:Endoscopic images showing Severe gastritis in fundus and antral polyp

Bone marrow aspiration indicated reactive marrow with focal megakaryocytic hyperplasia.

Fig 5:Bone marrow showing focal megakaryocytic hyperplasia

At 35 weeks and 5 days of gestation, the patient underwent an elective cesarean section,delivering of a single live baby weighing 2400 g. The postoperative course was uneventful.

Fig 6: Intraop images showing enlarged spleen

Splenomegaly is an important sign and challenging medical problem that associated with an underlying disease. In Ali et al. study tropical splenomegaly (52.7%), portal hypertension (26.3%), tuberculosis (8.8%), leishmaniasis (5.3%) and idiopathic thrombocytopenia (3.5%) were the identified causes for splenomegaly. Massive splenomegaly might cause some discomfort by the growing uterus[1] .

The complications of splenomegaly in pregnancy in terms of anemia, thrombocytopenia, increases the susceptibility to poor pregnancy outcomes. Anemia is a reported cause for stillbirth, preterm labor, preeclampsia and postpartum hemorrhage[4].The enlargement of the spleen during gestation presents a significant diagnostic challenge.The choice of delivery method is critical and often presents a dilemma.Normal labor and vaginal delivery may lead to spontaneous splenic rupture and subsequent massive hemoperitoneum, necessitating emergency surgical intervention. There is a risk of variceal bleeding because of repetitive Valsalva manoeuvre[5]  and hence some experts advise an elective caesarean section in those with high-grade varices[6,7]. Additionally, during cesarean delivery, the spleen is at risk of rupture while extracting the fetus, resulting in a potentially life-threatening situation requiring immediate management. These complexities complicate the obstetric management of patients with massive splenomegaly.

Maternal mortality is often attributed to extensive hemorrhage, concurrent hemorrhagic shock, and consumptive coagulopathy. Hemodynamic decompensation in the maternal system can precipitate a sudden decline in uteroplacental perfusion, resulting in the manifestation of "fetal distress" and eventually fetal demise [8].

It concludes that in cases with pancytopenia ,spleenomegaly during pregnancy, vigilant monitoring, prompt intervention, and a multidisciplinary approach are crucial to achieve positive outcomes for both the mother and the fetus.

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