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Acute unilateral leukemic hypopyon in an adult with relapsing acute lymphoblastic leukemia.

PURPOSE: To report acute unilateral hypopyon uveitis as an initial presenting feature of relapsing acute lymphoblastic leukemia (ALL) in an adult patient. DESIGN: Observational case report. METHODS: Anterior chamber paracentesis was performed in a 56-year-old male presenting with treatment-resistant unilateral hypopyon while in the remission phase of ALL. RESULTS: Examination of the aqueous humor aspirate revealed presence of malignant cells compatible with the previous bone marrow biopsy and subsequent spinal tap results. CONCLUSIONS: Atypical hypopyon uveitis can be an indication of relapsing ALL, even in adults. Prompt anterior chamber aspiration is required for the correct diagnosis and subsequent treatment.

Anterior Chamber↗

[Severe acute hepatic failure and haemophagocytic syndrome: cause or consequence?].

Haemophagocytic syndrome corresponds to an unconnected macrophagic activity with haemophagocytosis. We report the case of a haemophagocytic syndrome in a 49-year-old woman with initially a severe acute hepatic failure. This syndrome is probably underestimated in ICU patients. Haemophagocytic syndrome should be suspected in patients with fever and jaundice without infection.

Aged↗

Closure of the pericardium using synthetic bioabsorbable polymers.

BACKGROUND: Pericardial substitutes are known to ensure safer resternotomy at reoperation. A synthetic sheet made from expanded-polytetrafluoroethylene (e-PTFE) has been most commonly used as a pericardial substitute. The e-PTFE sheet, however, can induce severe inflammatory reaction and diffuse fibrosis. This study was designed to investigate the absorption rate and tissue reaction associated with two absorbable pericardial substitutes: a gelatin sheet and L-lactic acid-epsilon-caprolactone copolymer (L-C copolymer). In addition, e-PTFE sheet and autologous pericardium were used as controls. METHODS: Sixty dogs were divided into four groups of 15. In group A, a 3 x 3 cm segment of pericardium was excised, and the autologous pericardium was resutured. In group B, the pericardial defect was replaced with gelatin sheet. In group C, the defect was replaced with L-C copolymer sheet. In group D, the defect was replaced with e-PTFE sheet. For each group, the implanted membranes were retrieved at 2 weeks (n = 1), 4 weeks (n = 3), 12 weeks (n = 5), and 24 weeks (n = 6) after implantation. RESULTS: The e-PTFE sheet produced severe adhesions to the heart and pleura and a more prominent inflammatory reaction, as compared with the gelatin sheet. The absorbable pericardial substitutes were completely absorbed by 24 weeks after implantation, and were replaced with fibrous membrane. CONCLUSIONS: Gelatin sheet may involve less adhesion and a reduced inflammatory reaction compared with e-PTFE.

Absorbable Implants↗

Clinical presentations and complications of hairy cell leukemia.

HCL typically presents in middle-aged men, and is characterized by splenomegaly and cytopenias. Hepatomegaly may be present, but it usually is not a salient feature. Peripheral adenopathy is uncommon. Other organ manifestations occur, but are unusual. patients are now presenting with a less tumor burden, as a result of earlier diagnosis. Leukocytosis/lymphocytosis should suggest HCl variant. Infectious complications, which were common in the past and the major cause of death, have become rare in the era of purine analog therapy. Whether there is a true increased risk for second malignancies remains controversial.

Adult↗

Extramedullary relapse in a patient with acute promyelocytic leukemia: successful treatment with arsenic trioxide, all-trans retinoic acid and gemtuzumab ozogamicin therapies.

Acute promyelocytic leukemia (APL) is characterized by the presence of the t(15;17) translocation, resulting in the PML-RAR fusion protein. Standard treatment consists of the combination of all-trans retinoic acid (ATRA) with an anthracycline that results in complete remission (CR) rates in excess of 90%. Recently, several new agents have been shown to have clinical activity in APL. These include a liposomal formulation of ATRA (lipo-ATRA), and gemtuzumab ozogamicin (GO). Herein, we report a patient with APL who relapsed with extramedullary disease 2.5 years after lipo-ATRA therapy and was successfully treated with the sequence of A2O3, ATRA, and GO and we summarize our experience with patients with isolated extramedullary relapse in APL.

Aminoglycosides↗

Ocular adnexal granulocytic sarcoma as the first sign of acute myelogenous leukemia relapse.

PURPOSE: To report a case of granulocytic sarcoma involving the eyelids and caruncles after bone marrow transplantation. METHODS: Case report. A 30-year-old man with acute myeloid leukemia in remission developed multiple friable eyelid and caruncular lesions in addition to two cutaneous lesions on the chest wall and right axilla approximately 3 months after a successful autologous bone marrow transplant. RESULT: Pathologic examination was consistent with granulocytic sarcoma. CONCLUSION: This condition should be considered in the differential diagnosis of cutaneous or eyelid masses in patients with a history of leukemia.

Adult↗

Imaging of orbital lymphoproliferative disorders.

Lymphomas and leukemias account for a large portion of orbital tumors. Orbital lymphoma accounts for 55% of malignant orbital tumors in adults. Idiopathic orbital inflammatory pseudotumors are pathologic entities that often challenge ophthalmologists and radiologists. This article describes the MR and CT features of orbital lymphoma, leukemia, and some other lymphoproliferative disorders.

Adult↗

Methotrexate encephalopathy. A neuropathologic study.

The present report is a neuropathologic study of three patients who died from a severe form of necrotizing encephalopathy following intrathecal methotrexate therapy and craniospinal irradiation for acute lymphoblastic leukemia. During the early phase the lesion presented as multifocal coalescing areas of coagulation necrosis in the deep white matter. In the diseased areas the myelin was lost and the axons were severely swollen and fragmented. During the late stage the white matter was reduced to a thin gliotic calcified layer. The pathogenesis of this lesion is unclear, but the preferential involvement of the white matter with sparing of the cortical gray matter and basal ganglia makes it unlikely that direct chemical injury is the cause. The distribution of the lesion suggests a vascular disturbance due to endothelial injury caused by interaction of the methotrexate with tissue infiltrated by leukemic cells and altered by irradiation.

Brain↗

The indispensable role of microenvironment in the natural history of low-grade B-cell neoplasms.

Follicular lymphoma (FL) and B-cell chronic lymphocytic leukemia (B-CLL) are paradigmatic examples of lymphoid malignancies in which the relevant biological mechanisms are alterations in the control of apoptosis rather than an exaggerated proliferation. This explains why low-grade B-cell neoplasms still fail to be cured with current approaches. It is becoming increasingly clear that the defective apoptosis of FL and B-CLL has to be ascribed not only to intrinsic defects of the neoplastic cells, but also to extrinsic factors that influence their behavior. Malignant B cells retain the capacity to respond to microenvironmental signals, but have devised a monothematic responsiveness. They have a specific sensitivity to anti-apoptotic signals that favor their survival, whereas they seem to have become insensitive to pro-apoptotic signals. Bystander, nontumoral cells play a fundamental (though not sufficient) role both in the onset and in the progression of these diseases. The survival of leukemic cells appears to be dependent on direct cell-cell contacts. The localization of malignant B cells in bone marrow or neoplastic follicles is not a passive adhesion phenomenon but a crucial step for their survival. Bidirectional malignant lymphocyte-nontumoral cell interactions may lead to the amplification of a microenvironment able to inhibit the apoptosis of neoplastic B cells. The pressure of antigenic selection and the role of the tumor necrosis factor receptor family through the functional survival signal provided by CD40 together with the crippled death signal exerted by CD95 are new prominent characters on the stage.

Antigens, CD↗

Cerebellar granulocytic sarcoma in an infant with CD56+ acute monoblastic leukemia.

Granulocytic sarcoma (GS) is a form of extramedullary leukaemia (EML). The presence of the neural cell adhesion molecule (NCAM) on the surface of the blasts, which is recognized by the CD56 monoclonal antibody, enhances their propensity for tissue penetration. GS within the central nervous system (CNS), in particular within the cerebellum, is extremely uncommon. We review the literature and describe an infant with isolated cerebellar GS relapse, which antedated a CD56+ acute monoblastic leukaemia bone marrow (BM) relapse.

CD56 Antigen↗

CD56 expression predicts occurrence of CNS disease in acute lymphoblastic leukemia.

We examined the pre-treatment bone marrow samples from 200 consecutive adult patients with acute lymphoblastic leukemia (ALL) treated on various protocols at the University of Texas, M.D. Anderson Cancer Center between 1986 and 1998. Standard MFC techniques were used to determine CD56 expression on the leukemia blasts cells. The expression of CD56 was correlated with clinical characteristics at diagnosis, response to therapy, survival and disease-free survival. Blast expression of CD56 (> or = 20% of leukemic blasts) was seen in 16 (8%) of patients, with a median expression of 67% (range 20-99%). CD56 expression was associated with a higher incidence of central nervous system (CNS) disease at diagnosis (19% versus 4%; P=0.016). Incidence of CNS disease at any time was higher in patients with CD56+ disease (31% versus 14%; P=0.057). Among the 109 patients uniformly treated with the hyperCVAD regimen, CD56 expression was associated with a statistically significant higher incidence of CNS disease (33% versus 9%; P=0.026). CD56 expression in ALL is uncommon but may predict a higher risk for CNS disease. If these results are confirmed, CD56 expression could be used in combination with other high-risk features (e.g. lactate dehydrogenase (LDH), S-phase fraction, mature B-cell phenotype) to design a risk-oriented approach to CNS prophylaxis.

Adolescent↗

Pulmonary leukostasis mimicking pulmonary embolism.

We report a case of a 32-year-old woman who presented with shortness of breath and pleuritic chest pain, and mismatched perfusion defects on a ventilation-perfusion scan suspicious for pulmonary embolism. However, subsequent data revealed the diagnosis of acute myelogenous leukemia with hyperleukocytosis and associated pulmonary leukostasis. Unfortunately, the patient died despite urgent leukopheresis. Autopsy examination revealed extensive infiltration of leukemic cells in all major organs with no evidence of pulmonary embolism. This case highlights the clinical, radiographic and histologic features of pulmonary leukostasis, and reminds the clinician that not all ventilation-perfusion mismatching is due to thromboembolic disease.

Acute Disease↗