Case Presentation: A 79-year-old man presented with 4 months of progressive muscle weakness, shortness of breath, and anorexia associated with a 25 lb weight loss. His exam was notable for hypoxemia requiring 4 liters of oxygen, marked proximal muscle weakness, a left cranial nerve 6 palsy, and a petechial rash on his abdomen. Prior to admission, an extensive workup revealed elevated ESR and CRP but was otherwise unremarkable, and the patient was empirically started on prednisone for presumed polymyalgia rheumatica. On this admission, initial workup was notable for elevated ESR, CRP, and LDH, normal CK, normocytic anemia with nonspecific peripheral smear, and a faint lambda chain on serum protein electrophoresis. CT imaging of his chest/abdomen/pelvis was pertinent for diffuse pulmonary ground glass opacities, and a subsequent bronchoscopy revealed pneumocystis jirovecii pneumonia. Despite treatment he developed worsening respiratory failure requiring intubation and transfer to the ICU. Further workup with MRI brain/orbit/spine, lumbar puncture and bone marrow biopsy was unremarkable. A biopsy of the patient’s abdominal rash revealed atypical cellular invasion of small vessels within the dermis consistent with intravascular large B-cell lymphoma (IVLBCL). The patient received one round of a modified R-CHOP regimen, but remained delirious with high oxygen requirements. He was ultimately transitioned to comfort care and died shortly thereafter.
Discussion: IVLBCL is a rare type of non-Hodgkin’s lymphoma characterized by selective growth of neoplastic lymphocytes within the lumina of small vessels, particularly capillaries. Given the lack of nodal or marrow involvement and generally nonspecific symptoms, diagnosis is often delayed and prognosis can be poor. This patient’s course was consistent with the Western variant, which unlike its Asian counterpart is characterized by skin and neurologic involvement and a lack of detectable hematologic or marrow infiltration. The elevated LDH and ESR, anemia, and monoclonal serum component are all consistent with prior case reports. Therapy with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) appears to offer the highest chances of clinical response and 2-year overall survival.
Conclusions: Intravascular large B-cell lymphoma is a rare form of non-Hodgkin’s lymphoma characterized by localization of neoplastic lymphocytes to the lumina of small vessels. Its presentation is often nonspecific and should be considered in elderly patients with B symptoms, elevated LDH, and anemia, especially in the absence of lymphadenopathy, tumor or peripheral hematologic involvement. Diagnosis relies on tissue biopsy and there should be low threshold to sample any affected organ systems if the diagnosis remains in doubt.
To cite this abstract:Murphy R, Patil T, Mansoori J, Malkoski S, Misky G, Gottenborg E. The Answer Is Skin Deep. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 627. Journal of Hospital Medicine. 2015; 10 (suppl 2). https://www.shmabstracts.com/abstract/the-answer-is-skin-deep/. Accessed April 9, 2020.