Kocaeli Med J. 2024; 13(3): 220-223 | DOI: 10.5505/ktd.2024.87533

A Rare Cause of Pancytopenia: Splenic Cystic Lymphangiomatosis, Case Report and Literature Review

Ahmet Kürþat Soyer¹, Aziz Hakký Civriz², Büþra Yaprak Bayrak^2
1Department Of Internal Diseases, Faculty Of Medicine, Kocaeli University, Kocaeli, Turkey
²Department Of Pathology, Faculty Of Medicine, Kocaeli University, Kocaeli, Turkey

Only a very few cases of splenic lymphangiomatosis have been published in the literature to date. Herein,
we report the case of splenic lymphangiomatosis with unique characteristics. A woman in her 70s was
referred to us due to worsening of her pre-existing pancytopenia. Abdominal computed tomography scan
revealed an enlarged spleen (17 cm) and some hypodense lesions in the splenic parenchyma. These lesions
were evaluated as benign cystic lesions. Then, splenectomy was performed. The lesions were diagnosed as
lymphangiomatosis. Her thrombocytopenia, neutropenia, and lymphopenia were resolved postoperatively.
Her anemia regressed but persisted likely due to underlying chronic illnesses. Lymphangiomatosis should
be considered a rare cause in the differential diagnosis of hypersplenism and pancytopenia. Other potential
causes of hypersplenism and pancytopenia should also be thoroughly investigated to arrive at an accurate
diagnosis.

Keywords: splenic lymphangiomatosis, splenic cystic lymphangiomatosis, pancytopenia

---

Nadir Bir Pansitopeni Nedeni: Splenik Kistik Lenfanjiyomatozis, Vaka Sunumu ve Literatür taramasý

Ahmet Kürþat Soyer¹, Aziz Hakký Civriz², Büþra Yaprak Bayrak^2
1Kocaeli Üniversitesi Týp Fakültesi, Iç Hastalýklarý Ana Bilim Dalý, Kocaeli, Türkiye
²Kocaeli Üniversitesi Týp Fakültesi, Patoloji Ana Bilim Dalý, Kocaeli, Türkiye

Splenik lenfanjiyomatozis vakalarý literatürde çok az sayýda bildirilmiþtir. Daha önce bildirilmemiþ bir prezantasyonu ile splenik lenfanjiyomatozis vakasý sunuyoruz. 70'li yaþlarýnda kadýn hasta mevcut olan pansitopenisinin kötüleþmesi nedeniyle bize yönlendirildi. Batýn tomografisinde splenomegali (17 cm) ve dalak parankiminde öncelikle benign olduðu düþünülen kistik hipodens lezyonlar görüldü. Hastaya splenektomi yapýldý. Lezyonlar lenfanjiyomatozis olarak tanýmlandý. Trombositopenisi, nötrofpenisi ve lenfopenisi ameliyat sonrasý düzeldi. Anemisi geriledi ancak muhtemelen mevcut kronik hastalýklarý sebebiyle devam etti. Lenfanjiyomatozis, hipersplenizm ve pansitopeni ayýrýcý tanýsýnda nadir bir neden olarak göz önünde bulundurulmalýdýr. Ayýrýcý taný için hipersplenizm ve pansitopeninin diðer potansiyel nedenleri de detaylý bir þekilde araþtýrýlmalýdýr.

Anahtar Kelimeler: splenik lenfanjiyomatozis, splenik kistik lenfanjiyomatozis, pansitopeni

---

CITE