A previously healthy 23-year-old Caucasian woman presented to our emergency department with left-sided hemiplegia and a fever (39°C). She was later intubated and admitted to the ICU when she scored 7 out of 15 at the Glasgow Coma Scale. Upon admission, the patient underwent a brain computed tomography (CT) scan, which depicted a massive sylvian infarction associated with severe cerebral edema (Figure ); hence, she underwent a decompressive craniectomy.
A) Brain computed tomography scan depicting a large right-sided ischemic region and cerebral edema. B) Abdominal computed tomography scans revealing multiple enlarged lymph nodes and a retroperitoneal mass adjacent to the left psoas muscle.
Physical examination was unremarkable except for the presence of multiple palpable left-sided inguinal lymph nodes. Laboratory tests revealed leukocytosis (WBC: 17, 900 cells/mm3, 91% neutrophils), hemoglobin level at 10 gr/dL, elevated lactate deydrogenase at 356 IU/L and C-reactive protein at 197 and 8 mg/L.
Consequently, the patient underwent an abdominal CT scan that demonstrated multiple enlarged lymph nodes and a retroperitoneal mass adjacent to the left psoas muscle (Figure ). Finally, she underwent a transesophageal echocardiography that showed normal results.
Blood cultures taken from the patient were negative for bacterial, fungal and mycobacterial pathogens. Serologic tests for cytomegalovirus, herpes simplex virus, Epstein-Barr virus, Human Immunodeficiency Virus (HIV), tularaemia, Yersinia pestis, brucellosis, leptospirosis, Lyme disease, syphilis and Toxoplasma gondii were inconclusive. An examination of the patient's cerebrospinal fluid was negative. Results of the peripheral blood smear and bone marrow aspiration were not diagnostic.
Further laboratory tests for autoimmune disorders were also inconclusive. A diagnostic work up for inherited causes of thrombophilia such as protein C and S deficiency, antithrombin III deficiency, factor V Leiden gene mutation (associated with activated protein C resistance), prothrombin gene mutation, hyperhomocysteinemia, elevated lipoprotein (a) and polycythemia vera revealed no pathology. Furthermore, acquired prothrombotic states such as paroxysmal nocturnal hemoglobinuria, nephrotic syndrome, hyperviscosity disorders (Waldenstrom's macroglobulinemia, multiple myeloma) and sickle cell anemia were excluded conditions.
The patient underwent a CT-guided biopsy of her retroperitoneal mass, but no specific infection and/or malignancy was identified. Finally, a biopsy of an inguinal lymph node (Figure ) revealed the presence of PTCL-U. An immunochemistry analysis of the patient showed positive antibodies against CD3 (Figure ) and CD5, thus verifying the T-cell origin of her lymphoma. Meanwhile, she showed negative antibodies against CD10, CD30, CD57 and B-cell lymphoma 6. Her CD4-to-CD8 ratio was 4:1 for T-lymphocytes. Her Ki-67 was positive for 8% to 10% of the nuclei population.
Figure 2 A) Histological section showing multiple lymphocytes and zones of fibrosis consistent with an inguinal lymph node. A hematoxylin and eosin stain magnified 100 times was used. B) Histological section of the inguinal lymph node demonstrating positive antibodies (more ...)
The patient underwent chemotherapy consisting of cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP), but she developed multiple organ failure and died 26 days after her admission to the ICU. An autopsy revealed small periventricular and intraparenchymal mass infiltrations that caused multifocal occlusion of the small blood vessels.