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Intravascular Large B-Cell Lymphoma: A Rare Lymphoma with Bewildering Clinical Manifestations
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A3417 - Intravascular Large B-Cell Lymphoma: A Rare Lymphoma with Bewildering Clinical Manifestations
Author Block: Y. Wakwaya1, S. P. Malkoski2; 1Pulmonary Science and Critical Care Medicine, University Of Coloardo, Aurora, CO, United States, 2Univ of Colorado UCD Anshutz Campus, Aurora, CO, United States.
Intravascular Large B-Cell Lymphoma(IVLBCL) is a rare type of non-Hodgkin’s lymphoma characterized by the selective growth of neoplastic lymphocytes located in the lumen of small and medium sized vessels. Patients can present with a bewildering array of symptoms as any organ system can be involved. As a result, ~50% of cases are diagnosed incidentally on autopsy. Awareness of IVLBCL is needed as patients who do not receive a timely diagnosis and treatment usually succumb to the disease within 1 year.
Our patient was a 65-year-old Hispanic female who developed headache, nausea, and vomiting three weeks prior to admission. For this she had a computed tomography(CT) angiography head and neck which was normal. On the day of admission, she presented with new abdominal pain and platelet count of 36,000. After platelet transfusion, a lumbar puncture was performed, and ruled out meningitis. CT abdomen/pelvis was unremarkable. On hospital day three she became febrile and hypotensive. Repeat labs shows persistent thrombocytopenia, new anemia with hemoglobin of 5.8, shistocytes on peripheral smear, elevated creatinine of 1.4, and lactic acid of 7.4. On exam, she was tachycardic and somnolent. She was transferred to the ICU for she for possible thrombotic thrombocytopenia purpura. Head CT showed scattered foci of blood throughout the frontal lobes. Empiric plasma exchange was started. Hemolysis labs were unremarkable except for elevated LDH of 970. ADMATS13 level returned normal. She underwent random skin biopsy which showed IVLBCL.
The incidence of IVLBCL is 0.095 cases per million, and has a median age of 70 at diagnosis. The pathophysiology is thought to be related to tumor cells accumulating in the capillaries and post-capillary venules obstructing blood flow and resulting in ischemia. Proliferation of neoplastic lymphocytes is limited to the intravascular space because the malignant cells lack CD29 and CD54 surface ligands, which prevents translocation across the endothelium. In western countries, central nervous system(CNS) and skin are the most commonly involved organ systems. The most common CNS symptoms are cognitive impairment and paralysis. Neuroimaging may demonstrate almost any pattern of injury. Diagnosis is made by demonstrating lymphoma cells within small to medium sized vessels on biopsy. If IVBCL is suspected random deep skin biopsies may be performed although the sensitivity is low. Treatment is with anthracycline based chemotherapy, and retrospective reviews have estimated 3-year 30% overall survival with therapy. The addition of Rituximab has been associated with a higher two-year overall survival, and complete response.
Author Block: Y. Wakwaya1, S. P. Malkoski2; 1Pulmonary Science and Critical Care Medicine, University Of Coloardo, Aurora, CO, United States, 2Univ of Colorado UCD Anshutz Campus, Aurora, CO, United States.
Intravascular Large B-Cell Lymphoma(IVLBCL) is a rare type of non-Hodgkin’s lymphoma characterized by the selective growth of neoplastic lymphocytes located in the lumen of small and medium sized vessels. Patients can present with a bewildering array of symptoms as any organ system can be involved. As a result, ~50% of cases are diagnosed incidentally on autopsy. Awareness of IVLBCL is needed as patients who do not receive a timely diagnosis and treatment usually succumb to the disease within 1 year.
Our patient was a 65-year-old Hispanic female who developed headache, nausea, and vomiting three weeks prior to admission. For this she had a computed tomography(CT) angiography head and neck which was normal. On the day of admission, she presented with new abdominal pain and platelet count of 36,000. After platelet transfusion, a lumbar puncture was performed, and ruled out meningitis. CT abdomen/pelvis was unremarkable. On hospital day three she became febrile and hypotensive. Repeat labs shows persistent thrombocytopenia, new anemia with hemoglobin of 5.8, shistocytes on peripheral smear, elevated creatinine of 1.4, and lactic acid of 7.4. On exam, she was tachycardic and somnolent. She was transferred to the ICU for she for possible thrombotic thrombocytopenia purpura. Head CT showed scattered foci of blood throughout the frontal lobes. Empiric plasma exchange was started. Hemolysis labs were unremarkable except for elevated LDH of 970. ADMATS13 level returned normal. She underwent random skin biopsy which showed IVLBCL.
The incidence of IVLBCL is 0.095 cases per million, and has a median age of 70 at diagnosis. The pathophysiology is thought to be related to tumor cells accumulating in the capillaries and post-capillary venules obstructing blood flow and resulting in ischemia. Proliferation of neoplastic lymphocytes is limited to the intravascular space because the malignant cells lack CD29 and CD54 surface ligands, which prevents translocation across the endothelium. In western countries, central nervous system(CNS) and skin are the most commonly involved organ systems. The most common CNS symptoms are cognitive impairment and paralysis. Neuroimaging may demonstrate almost any pattern of injury. Diagnosis is made by demonstrating lymphoma cells within small to medium sized vessels on biopsy. If IVBCL is suspected random deep skin biopsies may be performed although the sensitivity is low. Treatment is with anthracycline based chemotherapy, and retrospective reviews have estimated 3-year 30% overall survival with therapy. The addition of Rituximab has been associated with a higher two-year overall survival, and complete response.
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