Waldenstrom’s Macroglobulinemia and Ascites: A Case Report

Abstract

Background

Waldenstrom’s disease is characterized by the presence of pathological changes in the B lymphocytes that are in the last stages of maturation. One characteristic of WM is the production of an abnormal high amount of IgM and hyper viscosity syndrome. The MW gets worse, symptoms such as fatigue, weight loss, night sweats, fever, recurrent infections and swollen lymph nodes develop in patients who have a known history of MGUS. In this clinical case, our patient without history of MGUS, presents for the first time for medical observation only for ascites and the presence of an interportocaval lymph node package. An atypical presentation of the disease that makes us reflect on the difficulty of making a diagnosis in the elderly patient and on pathogenetic hypotheses of ascites not yet explored.

Case Presentation

Seventy-three-year-old patient, hospitalized for the onset of ascites with sloping edema, diffuse left pulmonary opacification. At the ultrasound check, cava and portal vessels patent and of regular caliber, however with inversion of flow in correspondence with the right branch and of the door to the hilum, with a subdiaphragmatic retrocaval focus with a maximum diameter of about 3 cm, which cannot be better viewed. CT scan of the abdomen with confirmation of the presence of an interportocaval lymph node package. After evidence of the electrophoretic protein picture of a double component, probably monoclonal with positive urinary immunofixation for free K chains. IgM dosage equal to 2190 mg. Serum immunofixation practice that confirms the diagnosis of type B lymphoproliferative syndrome as per Waldenstrom’s disease, confirmed by bone marrow aspiration with morphological and flow cytometric study. Immediately begin chemotherapy with Bendamustine 120 mg. After 4 weeks of therapy with the reduction of IgM values, the patient no longer presented ascites.

Conclusion

This case has an unusual presentation of this disease and we could shed a new light on the possible pathogenesis of portal hypertension in Waldenstrom’disease.

Keywords:

• ascites
• portal hypertension
• Waldenstrom’s disease
• elderly

Abbreviations

MGUS, Monoclonal Gammopathy of undetermined significance; MW, Macroglobulinemia Waldenstrom; ECG, electrocardiography; BAV, atrioventricular block; PT, prothrombin time.

Consent of the Patient

The authors have the consent of the patient for the publication of the case. (CARE guidelines).The authors used the patient consent form (provided by the Taylor & Francis group, of which Dove Medical Press is a part) completed, signed and securely saved. Consent to publication has been obtained: the ethics committee of the ASL Napoli 1 approved the publication of the case. The authors are able to share it with the editors of the journal if requested.

Author Contributions

CB wrote the original manuscript and edited the manuscript, AC performed ultrasound examination, MG, PM, AG, AF, MGC, ML and PT managed the patient, MS provided pathological images with descriptions, PDM performed clinical supervision. All authors made a significant contribution to the work reported, whether that is in the conception, study design, execution, acquisition of data, analysis and interpretation, or in all these areas; took part in drafting, revising or critically reviewing the article; gave final approval of the version to be published; have agreed on the journal to which the article has been submitted; and agree to be accountable for all aspects of the work.

Disclosure

The authors report no conflicts of interest in this work.