Stručni rad
Unusual Presentation of Anaplastic Large Cell Lymphoma with Clinical Course Mimicking Fever of Unknown Origin and Sepsis: Autopsy Study of Five Cases
Marina B. Mosunjac
; Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Ga, USA
Bruce Sundstrom
; Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Ga, USA
Mario I. Mosunjac
; Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Ga, USA
Sažetak
Aim To describe a subset of cases with the unusual clinical and histomorphological
presentation of anaplastic large cell lymphoma (ALCL)
mimicking fever of unknown origin (FUO) and sepsis.
Methods A pathology database was searched using full term Systematized
Nomenclature of Medicine codes for ALCL to identify 23ALCL
cases from the period 1999-2006. Of those, five cases that did not have
a correct premortem diagnosis were further analyzed to elucidate the
reasons for delayed and incorrect pre-mortem diagnosis. The analyzed
data included clinical presentation, duration of symptoms, duration
of hospital stay, premortem presumed cause of death, white blood cell
count, platelet count, anion gap and blood pH, liver enzymes (alanine
aminotransferase, aspartate aminotransferase, lactate dehydrogenase,
alkaline phosphatase), lactate, coagulation tests (prothrombin time,
partial thromboplastin time, fibrinogen, D-dimers), microbiology cultures,
and radiology and surgical pathology reports. Autopsy reports
were reviewed for description of major gross findings, initial clinical
diagnosis, and cause of death.
Results Five fatal and pre-mortem unrecognized ALCL cases were
characterized by rapid decline, with histologic findings showing predominantly
extranodal involvement, intravascular lymphomatosis, and
hemophagocytosis. The cases were also characterized by unusual clinical
manifestations including a FUO, sepsis, and disseminated intravascular
coagulation-like picture, lactic acidosis, hepatosplenomegaly, and
absence of significant peripheral adenopathy.
Conclusions There is a distinct group of ALCLs with unique and specific
clinical, gross autopsy, and histopathologic findings. Recognition
of this clinical variant may facilitate early detection and potentially
timely diagnosis and therapy
Ključne riječi
anaplastic large cell lymphoma; autopsy; DIC; lactic acidosis; sepsis; hemophagocytosis and intravascular spread
Hrčak ID:
35103
URI
Datum izdavanja:
15.10.2008.
Posjeta: 1.141 *