ABSTRACT
Macrophage activation syndrome (MAS) is a rare immunologic syndrome, rapidly fatal in the absence of specific etiological treatment. It is defined by clinical, biological and cyto-histological criteria. Numerous etiologies have been described in MAS, the association with Hodgkin lymphoma (LH) is exceptional. We report the case of a young woman in whom a macrophage activation syndrome reveals a Hodgkin's lymphoma.
Subject(s)
Hodgkin Disease/diagnosis , Macrophage Activation Syndrome/etiology , Adult , Female , Hodgkin Disease/complications , Humans , Macrophage Activation Syndrome/diagnosisABSTRACT
Primary mediastinal non-Hodgkin's lymphoma(PMNHL) is a rare cancer. Exceptionally, it can be complicated by tracheÅsophageal fistulas, directly connecting the esophagus and the trachea and secondary to esophageal tumor or chemotherapy (hence the interest of our case). We report the case of a 24-year old Moroccan female patient, treated for primary mediastinal large B cell NHL revealed by dyspnÅa associated with dysphagia and alteration of general condition. The patient underwent chemotherapy but her health condition worsened after a second treatment due to the occurrence of recurrent pulmonary infections associated with cough during meals making swallowing impossible. Esogastroduodenal fibroscopy was performed which confirmed the diagnosis of tracheÅsophageal fistula. Outcome was marked by patient's death despite endoscopic stent placement and a good response to chemotherapy. Early discovery of tracheÅsophageal fistula in patients with PMNHL is essential because it enables the implementation of an appropriate treatment.
Subject(s)
Lymphoma, Large B-Cell, Diffuse/diagnosis , Mediastinal Neoplasms/diagnosis , Tracheoesophageal Fistula/diagnosis , Antineoplastic Agents/administration & dosage , Deglutition Disorders/etiology , Dyspnea/etiology , Fatal Outcome , Female , Humans , Lymphoma, Large B-Cell, Diffuse/drug therapy , Lymphoma, Large B-Cell, Diffuse/pathology , Mediastinal Neoplasms/drug therapy , Mediastinal Neoplasms/pathology , Stents , Tracheoesophageal Fistula/surgery , Young AdultABSTRACT
Aggressive natural killer cell leukemia (ANKL) is a disease entity within the spectrum of lymphoproliferative syndromes of NK cells. It is rare, preferentially affecting Asiatic people. It has been very rarely reported in the African population; hence the interest of our case. Our study involved a 19-year old female patient of Moroccan origin presenting with acute-onset bone marrow failure syndrome associated with tumor syndrome. The diagnosis of natural killer cell leukemia was retained based on microscopic and immunophenotypic study of the bone marrow. Patient's outcome was fatal; she died within 2 months of diagnosis due to septic shock. Patients with ANKL have a worse prognosis and the median survival time following diagnosis is, on average, two months. In the absence of a prospective study, no therapeutic guidelines have been developed.
Subject(s)
Anemia, Aplastic/diagnosis , Bone Marrow Diseases/diagnosis , Hemoglobinuria, Paroxysmal/diagnosis , Leukemia, Large Granular Lymphocytic/diagnosis , Shock, Septic/physiopathology , Bone Marrow Failure Disorders , Fatal Outcome , Female , Humans , Immunophenotyping , Leukemia, Large Granular Lymphocytic/pathology , Morocco , Young AdultSubject(s)
Hematopoietic Stem Cell Transplantation , Myopathies, Nemaline/therapy , Paraproteinemias/therapy , Adult , Fatal Outcome , Humans , Male , Morocco , Myopathies, Nemaline/complications , Myopathies, Nemaline/pathology , Paraproteinemias/complications , Paraproteinemias/pathology , Transplantation, AutologousABSTRACT
T-cell prolymphocytic leukemia is a lymphoid hematologic malignancy of T origin, mature, individualized well by the clinical, morphological, and immunophenotypic as well as cytogenetic aspects. We report the case of a 39-year-old man admitted in our department because of a spinal cord compression. The cerebrospinal fluid analysis had revealed an increased albuminemia level; MRI evoked a vertebral infiltration by a malignancy. Evolution was characterized by the appearance of a tumor syndrome and a peripheral lymphocytosis. Immunophenotyping and the lymph node biopsy concluded of a diagnosis of a T-cell prolymphocytic leukemia. To our knowledge, we report here the first case of T-cell prolymphocytic leukemia revealed by a spinal cord compression.