Simple headache revealed a rare lymphoma: Waldenstrom macroglobulinemia with unique markers: a case report and review of the literature

Abstract

Background Waldenstrom macroglobulinemia (WM) is a rare lymphoma with an incidence rate of 3 per million people per year, with approximately 1000 to 1500 new cases diagnosed each year in the USA. It is primarily seen in Caucasian males with a median age of 70 years old. Patients are most often asymptomatic, but WM can manifest itself with constitutional symptoms such as lethargy, bleeding, organomegaly, and neurological or fundoscopic abnormalities. WM is characterized by immunoglobulin M (IgM) monoclonal gammopathy, lymphocytic infiltration of bone marrow, and normocytic anemia due to bone marrow replacement. Case presentation Our patient is a Hispanic 67-year-old female that presents with one month of intermittent band-like bilateral headache accompanied by dizziness, light-headedness, nausea, and blurred vision. A thorough diagnostic workup was performed, including serum protein electrophoresis (SPEP) with serum immunofixation (SIFE) showing an M spike and IgM kappa. Bone marrow biopsy was significant for lymphoplasmacytic infiltration with nodular B cells (CD19+, CD20+, CD22+). Computerized Tomography (CT) imaging showed splenomegaly in the patient. Treatment was provided for hyperviscosity syndrome with plasmapheresis twice. The patient reported improvement of her symptoms and was then scheduled for chemotherapy. Throughout 7 months, our patient received multiple cycles of bortezomib, dexamethasone, and rituximab. While her symptoms improved her psychiatric status got progressively worse. Conclusion It is important not to neglect symptoms such as a headache, which may seem small, but could serve as a clue in the diagnosis of Waldenstrom's macroglobulinemia.

A rare case of left parapharyngeal space large schwannoma of unknown origin

“…Clinically, schwannomas produce few clinical symptoms and the main symptoms are usually due to the presence of a slow-growing mass, causing dysphagia, pain on palpation [ 1 ]. Schwannoma like other rare tumors can present with vague symptoms like headache and nausea [ 6 ] and rarely presenting ulceration; hence, its clinical diagnosis is difficult, and its definitive diagnosis is established by the histopathological study of the lesion [ 1 ].…”

“…Schwannomas can be divided into central or peripheral lesions, so differential diagnosis must be established with other tumors clinically similar such as neuromas, neurofibromas, granular cell myoblastoma, fibromas [ 6 ]. Radiological examinations, especially computed tomography (CT) or magnetic resonance imaging (MRI), are extremely important in its primary diagnosis, providing valuable information, showing a homogeneous, very well delimited and solid lesion with shooting pain on direct palpation [ 7 ].…”

Does Waldenstrom’s macroglobulinemia also cause bone destruction? A rare case report

“…WM is a rare indolent mature B-cell lymphoma that accounts for only 1% to 2% of hematological tumors. 1 It is common in elderly male patients in Europe and the United States and is rarely reported by Asians. Patients with WM can be asymptomatic, or IgM deposition and systemic symptoms can occur because of tumor cell infiltration, but there is no bone destruction response.…”

“…Waldenstrom’s macroglobulinemia (WM) is a rare malignant B-cell lymphoma that accounts for only 1% to 2% of hematological tumors and has an incidence of three cases per million people per year in the United States. 1 Because of the relatively low prevalence of WM in Asia, it has not been extensively studied. 2 The clinical manifestations of WM include thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and hyperviscosity syndrome.…”

Successful Implementation of Combined Treatment Methods for a Rare Recurrence of Waldenstrom Macroglobulinemia With Extramedullary Lesions

“…However, when analyzing foreign experience, there are descriptions of cases with lesions in the lungs, terminal parts of the colon, stomach, hard palate, and humerus diaphysis [ 3 , 6 , 9 - 14 ]. Particularly noteworthy is the damage to the central nervous system, the meninges, and the involvement of the cerebrospinal fluid in the pathological process, which forms the pathogenetic basis of the Bing-Neеl syndrome and is classified as a complication of WM [ 15 ].…”