Neuromyelitis optica spectrum disorder coinciding with hematological immune disease: A case report.

INTRODUCTION: Recently defined consensus criteria for the diagnosis of neuromyelitis optica spectrum disorders (NMOSD) allow establishing the diagnosis in patients without elevated AQP4-Ab and optic nerve involvement. According to the new extended definition, NMOSD is closely associated with extensive spinal cord inflammation occurring in the course of systemic autoimmune diseases as sarcoidosis or lupus erythematodes. NMOSD occurring in the course of hematological disease have not yet been reported in the literature. CASE REPORT: A 38 year old male subsequently developed thrombocytopenia, hemolytic anemia and agranulocytosis over a 23 month period. Three months after an episode of agranulocytosis, he noticed ascending sensory disturbances and progressive weakness of his legs. Within two days, symptoms worsened to give almost complete paraplegia and loss of sensation below a midthoracic level. MRI revealed signal hyperintensity and edema in T2-weighted sequences reaching from the 2nd cervical to the 9th thoracic vertebral body. Two years later, he developed a second episode with lesions in the spinal cord and periventricular areas of brain stem and thalamus. CONCLUSION: The relapsing time course and the topographical pattern of central nervous system lesions restricted to axial brain structures and the spinal cord fulfill the criteria that have recently been defined for AQP4-Ab-negative NMO-spectrum disease. Systematic studies on the association of hematological autoimmune phenomena and spinal cord disease are needed to clarify whether this coincidence is just a casual phenomenon or whether it points to a yet undiscovered but perhaps therapeutically interesting link of immunological mechanisms affecting both organ systems.

Antithyroid drugs induced agranulocytosis and multiple myeloma: case report and general considerations.

Antithyroid drugs as thionamides are largely used in the treatment of the thyrotoxicosis. Side effects were reported in less than 10% of the cases, especially hematological, hepatic or skin allergies. One of the most severe manifestations is agranulocytosis, probably based on an immune mechanism that is exacerbated by the presence of the thyroid autoimmune disease itself. If the presence of the severe leucopenia is actually an epiphenomenon of a preexisting hematological disturbance as multiple myeloma is debated. The myeloma may also be correlated with an autoimmune predisposition. We present the case of a 56 years old female patient diagnosed with Graves' disease, who developed agranulocytosis after 8 months of therapy with thiamazole. Two months after antithyroid drug's withdrawal, the granulocytes number increased and she received therapy with radioiodine. Two years later she came back for diffuse bone pain that turned out to be caused by a multiple myeloma, confirmed by bone marrow biopsy. It might be a connection between the severe form of leucopenia that the patient developed and the medullar malignancy.

Granulocytopenia associated with thymoma in a domestic shorthaired cat.

A 5-year-old, spayed female cat was referred because of a mass in the cranial mediastinum noted on thoracic radiographs. A thymoma was diagnosed following ultrasound and biopsy of the mass. Treatment was initiated with coarse-fraction radiation therapy using external-beam therapy (four fractions of 5 Gy). The mass responded, but granulocytopenia developed. Bone marrow examination showed a myeloid to erythroid ratio of approximately 1:1, with a left shift within the myeloid line. These findings, as well as the lack of toxic changes within the peripheral blood neutrophils, suggested immune-mediated destruction of peripheral granulocytes. Immune suppression with prednisone and cyclosporine was instituted. After 7 weeks, the neutrophil count returned to normal. The tumor was removed, and cyclosporine was reduced and eventually discontinued 3 weeks postsurgery.

Use of routine chest radiography in the evaluation of fever in neutropenic pediatric oncology patients.

Evaluation of febrile episodes in children who have become neutropenic during treatment for malignant disease has traditionally included radiography of the chest. It has been our impression that the yield of such examination is low. To test this hypothesis we reviewed all chest radiographs (CXRs) obtained in the above setting in our institution over the last 3 years. These radiographs were independently reviewed by two of us (R.C., J.F.). Sixty-one patients experienced 134 febrile neutropenic episodes for which a CXR was obtained. Only eight (6%) of these films revealed any abnormality. After careful review it was apparent that four of these radiographs did not represent a infectious process. Thus only four of 134 films (2.9%) indicated pulmonary infection as the probable cause of fever in the patient. All four of these patients had prominent respiratory signs or symptoms. Of patients who were febrile but without pulmonary signs/symptoms, only one of 49 had an abnormal radiograph. We feel that such a low yield (at most 2%) calls into question the routine practice of obtaining a CXR in the febrile neutropenic child who is otherwise asymptomatic.

Thickened cortical bones in congenital neutropenia.

Congenital neutropenia is an uncommon entity which may be familial and has a wide spectrum of clinical expression. Three sisters with the severe form of the disease, that suffered from recurrent infections which lead to their demise, are described. Review of their radiographs revealed the presence of cortical thickening of the bones. Although several syndromes with different bone abnormalities have been reported associated with neutropenia, the radiographic finding of thickened cortex in children with congenital neutropenia has not been previously described.

Computed tomography of neutropenic colitis.

Four patients developed neutropenic colitis as a complication of acute leukemia (three) or aplastic anemia (one). On computed tomography (CT), neutropenic colitis was characterized by cecal wall thickening (four) and pneumatosis (one). Intramural areas of lower density presumably reflected edema or hemorrhage. Clinical improvement and return of adequate numbers of functioning neutrophils coincided with decrease in cecal wall thickening on CT. Prompt radiologic recognition of this serious condition is crucial, since surgical intervention is probably best avoided.

Labeled cells in patients with malignancy.

The use of radioisotopes for cell labeling has been a major tool in hematology laboratory research. Chromium-51-labeling of hematologic cells and lymphocytes has been used for years to study the migration and sequestration of these cells in the spleen and other sites. The substantial recirculation of lymphocytes from blood into lymphoid tissue and back into blood is well described. Recently, new approaches for radiosotopic cell labeling have gained prominence in the investigation of various aspects of malignant diseases and in the clinical care of such patients. Isotopes such as indium-111 can be visualized with standard scanning techniques providing further information about the migration of normal and malignant cells has been discovered. In vivo studies have been performed with indium-111 in animals and humans, including comparisons of the migration of abnormal cells (malignant) and of lymphocytes to abnormal nodes. Evaluation and comparison of the migration of carcinoma cells, normal lymphoid cells, and malignant lymphoid cells in animals show markedly different patterns of distribution, which could have bearing on investigations of mechanisms of metastasis. In vivo human studies also have evaluated the migration patterns of lymphoid cells from patients with chronic lymphocytic leukemia and well-differentiated lymphoma, showing very different migrating behavior between these two polarities of a similar disease. These types of studies, while initially phenomenonologic, may provide a basis for a better understanding of these diseases. There are concerns about the use of an isotope such as indium-111 for the labeling of long-lived cells such as lymphocytes. Laboratory studies have demonstrated impaired cell function at high concentrations of radioactivity. Some workers have expressed concern about long-term changes in cells that recirculate. Others cite precedents of other long-term uses of isotopes, therapeutically, without detrimental effects. These concerns continue to be investigated. Finally, an area of much interest in the use of indium-111 is the labeling of granulocytes. This technique has been useful diagnostically, to localize infections. The major value in patients with malignancy, primarily with hematologic malignancies, is to evaluate the potential benefit of granulocyte transfusions. Many of these patients develop prolonged granulocytopenia and become infected, and granulocyte transfusions may become a therapeutic consideration.(ABSTRACT TRUNCATED AT 400 WORDS)

Donor-leukocyte imaging in granulocytopenic patients with suspected abscesses: concise communication.

Indium-111-labeled donor leukocytes were used for the detection of foci of suppuration in eight severely leukopenic patients with marrow suppression, either idiopathic or associated with chemotherapeutic regimens for leukemia. In three patients good correlation was found between the results of imaging and clinical signs or subsequent proof of inflammation. In the other five patients, in whom no evidence of localized suppuration occurred, no abnormal accumulations of radioactivity were demonstrable. Labeled donor leukocytes provide a method for locating suppurative foci in severely leukopenic patients in whom autologous leukocyte labeling is impractical.

Evaluation of infection in neutropenic patients with indium-111-labeled donor granulocytes.

Donor granulocytes, obtained by filtration leukophoresis, were labeled with In-111-oxine and injected into seven severely neutropenic patients. All patients were febrile, and presumed to have infection, although infection was eventually proved in only five. Excellent demonstration of infection was achieved in two patients, and fair localization in one. One negative scan became positive when repeated with sedimentation-derived cells from a different donor. This method may be useful in detecting infections in neutropenic patients.