ABSTRACT
BACKGROUND: Extranodal involvement is not unusual in mantle cell lymphoma (MCL) which accounts for 6% of non-Hodgkin's lymphomas. Simultaneous localization in the eyelid and in the breast, as observed in our case, is however exceptional. CASE REPORT: Chronic lymphoid leukemia (CLL) was suspected in a 71-year-old woman with asthenia, a cervical and axillary nodal enlargement and elevated lymphocyte count. Blood immunoflow cytometry analysis, occurrence of rapidly growing tumors involving the two breasts and eyelids and cytogenetic and molecular features led to the diagnosis of MCL. A very good partial remission was obtained with second-line polychemotherapy composed of cytarabin, cisplatin and dexamethasone, but lasted only 3 months after the end of 6 cycles. DISCUSSION: Primary breast and eyelid lymphomas are rare. Such localizations are exceptional in MCL and are signs of aggressive disease. Before extra-nodal involvement, MCL may simulate banal CLL. Therefore, systematic immunohistochemistry and if necessary molecular analysis are useful for early diagnosis of MCL. Prognosis is particularly poor. Conventional chemotherapy cannot provide cure of MCL and median survival is 48 months. For this reason, high-dose chemotherapy with stem cell graft has to be discussed in young patients. MCL is currently characterized by Bcl1 rearrangement, t(11-14) translocation and cyclin D1 overexpression among small B-cell lymphomas in recent REAL- and WHO-classifications.
Subject(s)
Breast Neoplasms/pathology , Eyelid Neoplasms/pathology , Lymphoma, Mantle-Cell/pathology , Aged , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Breast Neoplasms/diagnosis , Breast Neoplasms/therapy , Combined Modality Therapy , Eyelid Neoplasms/diagnosis , Eyelid Neoplasms/therapy , Female , Hematopoietic Stem Cell Transplantation , Humans , Immunohistochemistry , Lymphoma, Mantle-Cell/diagnosis , Lymphoma, Mantle-Cell/therapy , PrognosisABSTRACT
The pathogenic mechanisms of thrombosis during inflammatory syndromes are unknown. The aim of our study was to evaluate coagulation activation and fibrinolysis and to study an acquired protein S deficiency in 58 patients with an inflammatory syndrome of neoplastic (16), infectious (24) or systemic (18) origin and in 54 control subjects. The results indicated that coagulation activation, demonstrated by an increase in the prothrombin fragment 1+2, was present in patients with an inflammatory syndrome regardless of its origin. Free protein S, the only functionally active protein, was not reduced even though C4b-binding protein was increased in inflammatory syndromes. Thus, a prothrombotic state was found in inflammatory syndromes but is not explained by an acquired protein S deficiency. All except five patients had normal plasminogen activator inhibitor-1 levels.
Subject(s)
Acute-Phase Proteins/metabolism , Blood Coagulation/physiology , Inflammation/physiopathology , Peptide Fragments/metabolism , Protein S Deficiency/physiopathology , Protein S/metabolism , Prothrombin/metabolism , Adolescent , Adult , Aged , Aged, 80 and over , Case-Control Studies , Female , Fibrinolysis/physiology , Humans , Male , Middle Aged , Neoplasms/physiopathology , Plasminogen Activator Inhibitor 1/metabolism , Sepsis/physiopathology , SyndromeABSTRACT
A patient had a left atrial myxoma which was modified by flurbiprofen administration. The diagnosis was made 42 months after the first symptoms appeared. Flurbiprofen may have reduced interleukin-6 secretion by the tumor, leading to a delayed diagnosis.
