Subject(s)
Leukemia, Myelogenous, Chronic, BCR-ABL Positive/diagnosis , Nasopharyngeal Carcinoma/therapy , Nasopharyngeal Neoplasms/therapy , Neoplasms, Second Primary/diagnosis , Adult , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cisplatin/administration & dosage , Cisplatin/adverse effects , Combined Modality Therapy , Female , Fluorouracil/administration & dosage , Fluorouracil/adverse effects , Humans , Radiotherapy, AdjuvantABSTRACT
We report a case of Lymphocytic Lymphoma presenting with primary manifestation in the prostate. A 82 year-old man presented to emergency department with acute urinary retention. Digital rectal examination revealed a voluminous and firm prostate. Histology confirmed involvement of the prostate by small B Lymphocytic Lymphoma. The patient was treated with chlorambucil. Lymphocytic infiltration of prostate is a rare manifestation. However this may also be the first sign of an undiagnosed lymphoma. This observation shows that the prostatic lymphoma must be considered among the causes of low urinary retention.
Subject(s)
Leukemia, Lymphocytic, Chronic, B-Cell/diagnosis , Prostatic Neoplasms/diagnosis , Urinary Retention/etiology , Aged, 80 and over , Antineoplastic Agents, Alkylating/therapeutic use , Chlorambucil/therapeutic use , Humans , Leukemia, Lymphocytic, Chronic, B-Cell/drug therapy , Leukemia, Lymphocytic, Chronic, B-Cell/pathology , Male , Prostatic Neoplasms/drug therapy , Prostatic Neoplasms/pathologySubject(s)
Gaucher Disease/diagnosis , Adult , Aged , Female , Humans , Male , Middle Aged , Splenomegaly/etiologyABSTRACT
BACKGROUND: Gaucher disease (GD) is the most frequent lysosomal storage disorder; type 1 is by far the most common form. It is characterized by variability in age of onset, clinical signs and progression. It is usually diagnosed in the first or second decade of life with the appearance of bone pains, splenomegaly and thrombocytopenia, but the disease may be diagnosed at any age between 1 and 73 years. In the present study, we report 3 cases with late onset of GD in whom the disease was a surprise finding including one patient with Parkinson disease. This late onset is described as an adult form of Gaucher disease. FINDINGS: Molecular investigation showed mutational homogeneity in Tunisian adult patients suffering from GD. Indeed, all patients carry the p.N370S mutation: two patients at a homozygous state and one patient at compound heterozygous state. CONCLUSION: The p.N370S mutation presents a large variability in the onset of the disease and its clinical manifestation supporting the view that GD should be considered as a continuum phenotype rather than a predefined classification.
Subject(s)
Gaucher Disease/genetics , Glucosylceramidase/genetics , Mutation , Adult , Bone Diseases/genetics , Bone Marrow Examination , DNA Mutational Analysis , Female , Gaucher Disease/complications , Gaucher Disease/enzymology , Gaucher Disease/pathology , Gaucher Disease/therapy , Genetic Predisposition to Disease , Hepatomegaly/genetics , Heterozygote , Homozygote , Humans , Male , Middle Aged , Phenotype , Splenomegaly/genetics , Thrombocytopenia/genetics , TunisiaSubject(s)
Chromosome Deletion , Chromosomes, Human, Pair 5 , Myelodysplastic Syndromes/blood , Myelodysplastic Syndromes/drug therapy , Myelodysplastic Syndromes/genetics , Thalidomide/analogs & derivatives , Antineoplastic Agents/therapeutic use , Chromosomes, Human, Pair 5/genetics , Cytogenetic Analysis , Hematologic Tests , Humans , Lenalidomide , Remission Induction , Thalidomide/therapeutic use , Time Factors , Treatment OutcomeSubject(s)
Bone Marrow/pathology , Fractures, Spontaneous/diagnosis , Gaucher Disease/diagnosis , Magnetic Resonance Imaging , Tibia/pathology , Tibial Fractures/diagnosis , Biopsy, Needle , Femur/pathology , Fractures, Spontaneous/pathology , Gaucher Disease/pathology , Humans , Male , Middle Aged , Tibial Fractures/pathologyABSTRACT
Subacute necrotizing lymphadenitis or Kikuchi-Fujimoto disease is a benign pathological entity diagnosed chiefly in young adults. We report a case in a 20-year-old woman who presented with swollen lymph nodes and a fever. Disseminated lymphadenopathy with nodes measuring up to 6 cm in diameter was found upon physical examination. The erythrocyte sedimentation rate was elevated to 40 mm/h and the lactic dehydrogenase level to 593 IU/l. Findings were negative from serological tests for rubella, hepatitis B, hepatitis C, HIV, and toxoplasmosis, as well as from tests for tuberculosis. A biopsy of a cervical lymph node showed nonsuppurative necrosis, karyorrhexis, and a marked histiocytic reaction consistent with Kikuchi-Fujimoto disease. No treatment was given, and a full recovery was achieved within 3 months. In this patient, the disseminated lymphadenopathy and constitutional symptoms strongly suggested a hematological malignancy and more specifically a lymphoproliferative disease.
Subject(s)
Histiocytic Necrotizing Lymphadenitis/diagnosis , Histiocytic Necrotizing Lymphadenitis/pathology , Adult , Blood Sedimentation , Female , Histiocytes/pathology , Histiocytic Necrotizing Lymphadenitis/blood , Humans , Lymph Nodes/pathology , NecrosisABSTRACT
INTRODUCTION: Glanzmann's thrombasthenia (GT) is a rare congenital thrombopathy, with a recessive autosomal transmission. We present here the genealogic study of a series of patients suffering from GT. PATIENTS AND METHODS: This is a retrospective study about all the GT patients treated in Sfax hematology department during 18 years. Final diagnosis was established by agregometry. Genealogic study was based on clinical history. RESULTS: 17 cases of GT from 11 families from the south of Tunisia wen collected. The disease was paricularly frequent in the region of Moulares-Gafsa (7 patients). The percentage of consanguinity was also very high (82%), with a third degree consanguinity of 86%. Family investigation revealed 6 previously unknown cases, and 10 deaths subsequent to hemorrhagic manifestations. CONCLUSION: The high rate of consanguinity, the absence of clinical or biological manifestations in the parents, and the ratio of ill to normal subjects in the same family which was about one to four, are suggestive of an autosomal recessive mode of transmission.
Subject(s)
Consanguinity , Genetic Predisposition to Disease , Thrombasthenia/genetics , Adolescent , Adult , Child , Female , Humans , Male , Pedigree , Retrospective Studies , Thrombasthenia/epidemiology , Tunisia/epidemiologyABSTRACT
Gaucher's disease is an uncommon inborn recessive autosomal disease, due to a deficient activity of the lysosomal enzyme beta glucocerebrosidase. This disease is usually diagnosed in the first or second decade of life with the arising of bone pains, splenomegaly and hemorragic manifestations due to thrombocytopenia. When the enlarged spleen is not evident, or after splenectomy, patients may be mis-identified as having Gaucher's disease. We present here two cases of elderly patients aged 70 and 46 years respectively, in whom the disease was a surprising finding of bone marrow examination, during check up for pancytopenia.
Subject(s)
Gaucher Disease/diagnosis , Age Factors , Aged , Female , Humans , Male , Middle AgedABSTRACT
In this report, we studied the frequency, the types and the prognosis value of the Durie and Salmon's classification of radiological bone lesions in multiple mycloma. Our study concerned 52 patients presenting multiple myeloma, defined according to South West Oncology Group criteria, collected during nine years (1988-1996). Radiological anomalies were noted in 89% of the cases. When we compared the medial survival and the 5 years survival rate in the different groups, we found a statistically significant difference with a better survival for groups 0,1,2 considered all together as compared to group 3 patients (p = 0.0155), (71% versus 37%). According to our series, prognostic value is obviously significant when comparing groups 0, 1, and 2 to group 3.