1/10. Sudden bilateral deafness from hyperleukocytosis in chronic myeloid leukemia.Sudden-onset bilateral deafness as a clinical manifestation of hyperleukocytosis in chronic myeloid leukemia (CML) is a rare occurrence. We found only 27 clinical descriptions in 16 published papers. In this work, the authors present a review on deafness in CML and describe a new case with prominent hyperleukocytosis, where the neurological findings suggest slowing of the circulation through small blood vessels in the brainstem as the cause of deafness. The evolution was good after treatment. To our knowledge, this is the second case documented with electrical auditory brainstem-evoked potentials and the first with magnetic resonance imaging.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/10. The neurological masquerade of intravascular lymphomatosis.BACKGROUND: Intravascular lymphomatosis (IVL) is an uncommon systemic disease characterized by occlusion of small vessels by malignant lymphomatous cells. central nervous system involvement usually presents as subacute encephalopathy, dementia, seizures, or multifocal cerebrovascular events. OBJECTIVE: To increase awareness about IVL, an uncommon cause of neurological disease. DESIGN: This is a retrospective case series of 8 pathologically proved cases of IVL with neurological disease. patients were part of a pathological series collected between April 1962 and October 1998 at indiana University School of medicine and the Armed Forces Institute of pathology, washington, DC. SETTING: Neurological and neuropathological examinations were performed at tertiary referral hospitals. patients: Eleven patients were diagnosed pathologically as having IVL, but 3 were not included in this evaluation because of a lack of appropriate clinical information. Of the final sample (n = 8), there were 4 men and 4 women (mean /- SD age, 62.9 /- 9.9 years). RESULTS: All 8 patients had focal neurological deficits, 7 had encephalopathy or dementia, 5 had epileptic seizures, and 2 had myelopathy. death occurred at a mean of 7.7 months (range, 1-24 months) after the onset of symptoms. All patients had elevated cerebrospinal fluid protein levels, 4 had pleocytosis, and 2 had an elevated IgG level in their cerebrospinal fluid. Of the 4 patients who underwent a brain biopsy, 1 was diagnosed as having IVL before death. CONCLUSIONS: Intravascular lymphomatosis is an uncommon disease with a myriad of potential neurological manifestations. diagnosis requires a high index of suspicion and a pathological examination. If diagnosed early, aggressive chemotherapy is potentially curative, although the overall prognosis remains dismal.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/10. Acute myocardial infarction as the presenting symptom of acute myeloblastic leukemia with extreme hyperleukocytosis.This case report deals with an unusual leukostatic complication in a 56-year-old woman with acute myeloblastic leukemia (AML) and extreme hyperleukocytosis (316 x 10(9)/L) who presented with acute myocardial infarction (MI). After leukopheresis the patient achieved hemodynamic stabilization and rapid neurologic recovery of encephalopathy that had also developed after the infarction. Considering the central role of WBC in the remodeling of post MI myocardial tissue, it was obvious that administration of chemotherapy with its subsequent inevitable pancytopenia could impose an increased risk for further cardiac complications including myocardial rupture. Nevertheless, cytarabine-based induction chemotherapy was initiated 3 days after admission, and she achieved prolonged complete remission. coronary angiography disclosed segmental atherosclerosis, but the only significant obstruction was in the right coronary artery. The patient died with relapsed leukemia 7 years later without recurrence of any cardiac symptoms or signs. autopsy disclosed segmental coronary atherosclerosis involving the LAD (60% obstruction), suggesting that atherosclerosis was a predisposing risk factor. Additional compromise to blood perfusion due to leukostasis had led to this unusual complication of AML involving a major vessel. This is the first documented case of leukostasis causing coronary artery occlusion as well as the first report of successful induction chemotherapy for AML during a myocardial infarction.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/10. Chronic lymphocytic leukemia presenting with extreme hyperleukocytosis and thrombosis of the common femoral vein.Very few case reports dealing with chronic lymphocytic leukemia (CLL) and hyperleukocytosis have been reported in the medical literature and none with venous thrombosis as a complication. Here, we describe a 73-year-old woman who presented with newly diagnosed CLL, leukostasis, and hyperleukocytosis (2000 x 10(9)/l), affecting the respiratory and nervous system. In addition, she also had deep vein thrombosis (DVT). Although hypercoagulability and thrombosis are well-described phenomena in solid tumors and in myeloproliferative neoplasms, CLL is generally not associated with an acquired coagulopathy. We hypothesize that in our patient the extreme number of circulating lymphocytes resulted in an abnormal accumulation of lymphocytes possibly causing stasis and occlusion of a larger vessel, which resolved after leukopheresis. The patient has since been successfully maintained with chemotherapy. We conclude that leukopheresis should be considered as the therapy of choice in CLL patients presenting with major complications of leukostasis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/10. A 19-year-old man with leucocyte adhesion deficiency. in vitro and in vivo studies of leucocyte function.We describe a male patient with leucocyte adhesion molecule deficiency (LAD) of moderate phenotype. Although diagnosis was made only 2 years before his death, the patient survived until 19 years of age. This enabled us to perform a number of novel investigation, both in vivo and in vitro, relating to his leucocyte biology. monocytes cultured in vitro matured into morphologically normal, phagocytically capable macrophages, which were able to recognize aged 'apoptotic' neutrophils. By injection of radiolabelled autologous neutrophils we demonstrated a prolonged neutrophil half-life, but normal margination, de-margination on exercise, and splenic pooling. Neutrophil adherence in vitro to vascular endothelium was normal. Histological examination of the patient's lungs at post-mortem showed intravascular aggregation of polymorphonuclear leucocytes but a paucity of cells in the interstitium and alveolar spaces. These findings indicate that the peripheral blood leucocytosis commonly observed in these patients may be due to prolonged intravascular neutrophil survival, and suggest that CD11/18 molecules have an important role in facilitating neutrophil emigration from blood vessels at sites of inflammation.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/10. Life threatening sickle chest syndrome treated with extracorporeal membrane oxygenation.A young West Indian woman with established sickle cell disease developed a severe episode of sickle chest syndrome. Conventional treatment including exchange transfusions and mechanical ventilation was to no avail, and an infusion of epoprostenol also failed to halt her worsening condition. When her arterial oxygen tension (PaO2) had fallen to 6.5 kPa (49 mm Hg) extracorporeal membrane oxygenation was instituted. Within two days her PaO2 was greatly improved (maximum 11.6 kPa; 87 mmHg), and by the sixth day pulmonary vascular resistance was reduced and angiography showed reperfusion of many vessels. The patient recovered and six months later showed a transfer factor close to the predicted range and normal spirometric values. extracorporeal membrane oxygenation should be considered for severe sickle chest syndrome when conventional methods of artificial ventilation fail.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/10. Acral lividosis--a sign of myeloproliferative diseases. Hyperleukocytosis syndrome in chronic myelogenous leukemia.Acral ischemia with lividity is a well-described dermatologic sign in the myeloproliferative diseases polycythemia vera and essential thrombocythemia. It has not previously been reported as a sign of chronic myelogenous leukemia (CML). We suggest the term acral lividosis to describe this clinical entity in patients with any myeloproliferative disease. We propose that the pathophysiology of acral lividosis in CML involves occlusion of small blood vessels of the skin by large, nondeformable myeloblasts, a process that has been shown histologically to occur in other organs in patients with CML. This process, called leukostasis, occurs in patients with CML who have over 50.0 X 10(9)/L (50,000/mm3) circulating myeloblasts. patients manifest cardiorespiratory and central nervous system compromise, a clinical constellation known as the hyperleukocytosis syndrome. Acral lividosis occurred in a patient with CML in whom nearly every organ demonstrated leukostasis on autopsy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/10. Sweet's syndrome in infancy.A 3-month-old Japanese girl developed tender erythematous elevated plaques on her face and buttocks with fever up to 38 degrees C. histology showed a perivascular neutrophil infiltrate with nuclear debris in the dermis. endothelial cells were swollen but no hyalinization of the vessel wall was observed. The lesions recurred several times and responded little to antibiotics but dramatically to oral corticosteroid. We diagnosed her skin condition as Sweet's syndrome in infancy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/10. arthritis with an inflammatory dermatosis resembling Sweet's syndrome. Report of a unique case and review of the literature on arthritis associated with the inflammatory dermatoses.A patient with a unique case of chronic episodic arthritis coincident with flares of acneform, pustular, nodular and ulcerating skin lesions was observed over a five-year period. This patient and a review of the literature on arthritis associated with the inflammatory dermatoses provide evidence which may interrelate several of these nosologically confusing skin conditions, e.g., the family of leukocytoclastic angiitides with the newly posited acute febrile neutrophilic dermatosis of Sweet. Systemic manifestations and a variety of acneform, pustular, nodular and ulcerating cutaneous lesions in the inflammatory dermatoses are best explained by small vessel involvement, with individual syndromes being determined by the type and degree of vascular change. Perivascular neutrophilic infiltration is the unifying histologic feature of these small vessel diseases. neutrophil infiltration differentiates these entities, and our patient, from the histologically nonspecific inflammations of the skin, e.g., Behcet's disease and pyoderma gangrenosum, which, although capable of causing identically appearing skin lesions, consist predominantly of lymphocytic dermal infiltrates even in the earlier stages. It appears important to recognized these morphologically varied acute inflammatory dermatoses with perivascular neutrophilic infiltration in view of their systemic features and the dramatic efficacy of corticosteroid therapy.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
10/10. Profound cerebrospinal fluid pleocytosis and Froin's syndrome secondary to widespread necrotizing vasculitis in an hiv-positive patient with varicella zoster virus encephalomyelitis.Demonstration of the direct involvement of cranial blood vessels by varicella zoster virus (VZV) is facilitated by immunohistochemistry (IHC), in situ hybridization (ISH) and polymerase chain reaction (PCR) techniques. The extent to which an inflammatory vasculitis serves as the pathogenic mechanism for VZV encephalomyelitis (VZVE) is still, however, debated. Most VZVE patients are immunocompromised and show little inflammation, either pre-mortem in cerebrospinal fluid (CSF) or at autopsy. We describe an hiv-positive patient with a moderately depressed CD4 count (304) who presented with massively elevated CSF protein (1800 mg/dl), bloody CSF and pleocytosis (1300 white blood cells (WBC)/mm3). His CSF was positive for VZV dna by PCR. He was treated with acyclovir and foscarnet, but died. At autopsy, an unusually widespread, inflammatory, transmural vasculitis caused by VZV affected meningeal vessels at virtually all brain stem and spinal cord levels, causing multiple subpial hemorrhages and necrosis. Virus dna in multiple areas of brain, brainstem and spinal cord was readily revealed by PCR, but not by the presence of viral inclusions, IHC or ISH. This case, with a clinically confusing presentation for VZVE, illustrates the extensive, albeit infrequent, degree of necrotizing vasculitis and CSF abnormalities that VZV is capable of producing. Antiviral therapy may have inhibited VZV genome replication and subsequent antigen production, resulting in negative ISH and IHC studies, but generated increased VZV genomic fragments that were detectable by the more sensitive PCR technique.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
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