ABSTRACT
Central nervous system (CNS) involvement in acute promyelocytic leukemia (APL) is rare and tends to be seen mostly following treatment with all-trans retinoic acid (ATRA), due to prolonged patient survival and poor penetration of the drug in the CNS. At least 10% of extramedullary relapses in APL involve the CNS, and associated factors include an increased age, the BCR isoform, the development of differentiation syndrome, a high white cell count at presentation and hemorrhage into the CNS during induction therapy. We present the case of a patient with high-risk APL, CD56+, CD2+ in whom a CNS relapse was diagnosed through the presence of a PML/RARα rearrangement on PCR of the cerebrospinal fluid (CSF).
Subject(s)
Central Nervous System Neoplasms/diagnosis , Leukemia, Promyelocytic, Acute/diagnosis , Oncogene Proteins, Fusion/cerebrospinal fluid , Adult , Antineoplastic Agents/therapeutic use , CD56 Antigen/genetics , CD56 Antigen/metabolism , Central Nervous System Neoplasms/cerebrospinal fluid , Central Nervous System Neoplasms/drug therapy , Fatal Outcome , Humans , Leukemia, Promyelocytic, Acute/cerebrospinal fluid , Leukemia, Promyelocytic, Acute/drug therapy , Male , Neoplasm Recurrence, Local/cerebrospinal fluid , Neoplasm Recurrence, Local/drug therapy , Neoplasm Recurrence, Local/pathology , Oncogene Proteins, Fusion/genetics , Polymerase Chain Reaction , Tretinoin , fms-Like Tyrosine Kinase 3/genetics , fms-Like Tyrosine Kinase 3/metabolismABSTRACT
BACKGROUND: The aim of this work was to estimate the significance of a dynamic study performed during the first 20 minutes after autologous (111)In-oxinate-platelets injection in patients with chronic immune thrombocytopenic purpura (ITP). Two hypotheses were tested: a) dynamic study indicates the place of platelet sequestration; b) dynamic study reflects the quality of platelet separation and labelling procedure. MATERIAL AND METHODS: Thirty-nine persons were investigated: 25 with shortened platelet life span (ITP), and 14 with normal platelet life span (6 healthy subjects and eight patients with myelodysplastic syndrome--MDS). Platelet blood count on the day of platelet labelling, general yield of platelet labelling (GYL), differential yield of platelet labelling (DYL), platelet life span, dynamic study with initial platelet accumulation in the liver (IPAL), sequential static study for determining the platelet sequestration index (SI) and platelet sequestration site (SS) were investigated. RESULTS: Two types of labelled platelet kinetics were determined in both groups of patients: IPAL < 20% and IPAL > 20%. A statistically significant difference in GYL and DYL was noted between the patients with IPAL < 20% and IPAL > 20%. No significant difference was registered in platelet blood count, life span, SS and SI between the two groups of patients. Both yields of platelet labelling were higher in the group with IPAL < 20%. There was no correlation between IPAL and platelet SI, or between IPAL and platelet SS. CONCLUSIONS: Dynamic study with (111)In-platelets cannot predict platelet sequestration site in ITP patients, but it is a useful and sensitive method of platelet labelling procedure quality control.