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Abstract

Background: Plasmablastic lymphoma (PbL) has been recognized by the World Health Organization (WHO) classification as a distinct subtype of diffuse large B-cell lymphoma (DLBCL), which are characterized with a strong expression of plasma cell markers instead of conventional B-cell markers. Although strongly associated with HIV-positive patients, it has also been commonly described in patients with solid organ transplants as well as immunocompetent patients. Plasmablastic lymphoma has shown to have an increase affinity for the oral cavity, followed by digestive tract. Furthermore, tumor involvement of the digestive tract accounts for around 20% of plasmablastic lymphomas. Although mainly found in the oral cavity, a few cases were documented to affect the gastrointestinal tract, and of those, only 3 cases were found to involve the esophagus, and we present the 4th case in a 48-year-old HIV positive male.

Case Presentation: A 48-year-old male with past medical history of hypertension, alcohol dependence, chronic macrocytic anemia, with a smoking history presents to the emergency department with multiple complaints including severe weight loss, poor appetite, fatigue, and nonproductive cough with sinus drainage for the past month. According to the patient he has lost around 50 pounds in the span of 4 to 5 months and has been complaining of early satiety for the past 2 months. Patient admits to drinking more than 1 pint of hard liquor a day for the past 4 years and has been smoking half a pack a day for the past 30+ years. Patient is a homosexual, has multiple partners, and does not use protection. During his visit to the emergency department patient had 2 episodes of vomitus with large bright red blood, patient denies any history of varices or variceal bleeding. Computed tomography (CT) scan of the abdomen/pelvis without contrast was initially done and was negative for any acute pathology. Patient was resuscitated with intravenous (IV) fluids and 2 units of packed red blood cells and was started on octreotide drip for possible variceal bleed. Patient subsequently underwent EGD for his hematemesis and a friable ulcerated circumferential mass lesion was seen in the distal esophagus extending from 28 to 40 cm from the incisors with active oozing that was seen from the mass. Multiple biopsies were obtained and sent to pathology. Patient was tested for HIV which was positive with a viral load of 219,000 cpy/mL, with appropriate HIV 1 genotyping. Pathologic analysis of esophageal biopsy showed a diffuse perforation of the large, atypical cells associated with brisk mitotic activity consistent with plasmablastic lymphoma. Patient was started on chemotherapy with dose adjusted V-EPOCH and highly active antiretroviral therapy (HAART) with Truvada and Dolutegravir.

Conclusion: Esophageal plasmablastic lymphoma is an extremely rare and aggressive subtype of diffuse large B-cell lymphoma and is most commonly found in HIV-positive patients. Disease awareness and early detection is warranted especially those with risk factors such as smoking, alcohol use, and HIV with concurrent B symptoms, as 55% of extra-oral PbL patients were found to be at stage IV during diagnosis.

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A Case of Esophageal Plasmablastic Lymphoma with Literature Review

Background: Plasmablastic lymphoma (PbL) has been recognized by the World Health Organization (WHO) classification as a distinct subtype of diffuse large B-cell lymphoma (DLBCL), which are characterized with a strong expression of plasma cell markers instead of conventional B-cell markers. Although strongly associated with HIV-positive patients, it has also been commonly described in patients with solid organ transplants as well as immunocompetent patients. Plasmablastic lymphoma has shown to have an increase affinity for the oral cavity, followed by digestive tract. Furthermore, tumor involvement of the digestive tract accounts for around 20% of plasmablastic lymphomas. Although mainly found in the oral cavity, a few cases were documented to affect the gastrointestinal tract, and of those, only 3 cases were found to involve the esophagus, and we present the 4th case in a 48-year-old HIV positive male.

Case Presentation: A 48-year-old male with past medical history of hypertension, alcohol dependence, chronic macrocytic anemia, with a smoking history presents to the emergency department with multiple complaints including severe weight loss, poor appetite, fatigue, and nonproductive cough with sinus drainage for the past month. According to the patient he has lost around 50 pounds in the span of 4 to 5 months and has been complaining of early satiety for the past 2 months. Patient admits to drinking more than 1 pint of hard liquor a day for the past 4 years and has been smoking half a pack a day for the past 30+ years. Patient is a homosexual, has multiple partners, and does not use protection. During his visit to the emergency department patient had 2 episodes of vomitus with large bright red blood, patient denies any history of varices or variceal bleeding. Computed tomography (CT) scan of the abdomen/pelvis without contrast was initially done and was negative for any acute pathology. Patient was resuscitated with intravenous (IV) fluids and 2 units of packed red blood cells and was started on octreotide drip for possible variceal bleed. Patient subsequently underwent EGD for his hematemesis and a friable ulcerated circumferential mass lesion was seen in the distal esophagus extending from 28 to 40 cm from the incisors with active oozing that was seen from the mass. Multiple biopsies were obtained and sent to pathology. Patient was tested for HIV which was positive with a viral load of 219,000 cpy/mL, with appropriate HIV 1 genotyping. Pathologic analysis of esophageal biopsy showed a diffuse perforation of the large, atypical cells associated with brisk mitotic activity consistent with plasmablastic lymphoma. Patient was started on chemotherapy with dose adjusted V-EPOCH and highly active antiretroviral therapy (HAART) with Truvada and Dolutegravir.

Conclusion: Esophageal plasmablastic lymphoma is an extremely rare and aggressive subtype of diffuse large B-cell lymphoma and is most commonly found in HIV-positive patients. Disease awareness and early detection is warranted especially those with risk factors such as smoking, alcohol use, and HIV with concurrent B symptoms, as 55% of extra-oral PbL patients were found to be at stage IV during diagnosis.

 

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