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P102 Lung involvement by non-Hodgkin anaplastic large cell lymphoma (ALCL)
Abstracts
/ European
Journal
of Internal
disorders. Its occurs most frequently in elderly patients. Anti FVIII inhibitors have been reported in autoimmune diseases, lymphoproliferative disorders, cancers, post-partum, drug reactions and after transfusions. No predisposing factor was found in the reported cases, same as for 30% of patients. Bleeding is often severe, occuring spontaneously or after minor trauma. Overall mortality is 20%. Minor bleeding may require only local treatment or desmopressin. Steroids alone are useful in 30% of cases. Recombinant activated FVII or ACPP directly active the factor X. Serious bleeding may need high doses of FVIII. Other immunosuppressive drugs, plasmapheresis, intravenous immunoglobulines and rituximab have also been used. There is no consensus on the best therapeutic approach but it is always an expensive therapy. The two reported cases are particular because of the very old age of the patients. Mortality is probably high in this age group but there is no study on acquired haemophilia in very old patients.
Ploo Paroxysmal nocturnal hemoglohinuria and myelodysplasia A. Ruellan, T. Kestler, B. List, S. Scheringer, 0. Mainz, B. Schoenberger, F. Paul, M. Burkert (Dobel, Ingolstadt, D) Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired stem cell disorder due to a mutation of the X-linked phosphatidylinositol glycan complementation class A (PIG-A) gene. Mutated clones expresse a severe deficiency in all surface glycosylphosphatidylinositol (GPl)-anchored proteins (decay-accelerating factor=CD 55, membrane inhibitor of reactive lysis=CD 59, CB-binding protein=C8 bp), leading to an increased complement-mediated lysis. This disease presents in most of cases with episodes of hemoglobinuria and life-threatening venous thromboses. Its is associated with an aplastic anaemia (AA) in 30% of cases. Other haematological disorders such as myelodysplasia may be found but more rarely. We report a case of PNH with a myelodysplasia in a 37 year patient. In 1997 a PNH was diagnosed with a megaloblastic anaemia and a moderate transfusion requirement. He had no other complications of the PNH. In April 2002 he was admitted because of tiredness, with a megaloblastic (131 ~3) anaemia (haemoglobin 7 g/dl) without hemolysis. Platelets and leucocytes were normal. The proteins CD 55 and CD 59 were negative on red cells. The bone marrow aspiration was compatible with an hypoplastic myelodysplasia (refractory anaemia subtype). The bone marrow caryotype was normal. His state improved with erythrocytes transfusion. The relationship between myelodysplasia and PNH is not clear. The development of PHN clones requires a mutation of haematopoietic stem cells and an hypoplastic bone marrow environment, such as in AA or myelodysplasia. It has also been reported that the presence of a minor population of PNH-type cells in myelodysplasia is associated with a better prognosis, probably because of immunological mechanisms. PNH cells may escape damage and apoptosis because they have lost most of their ability to produce GPI. In the diagnosis procedure of a bone marrow failure it is suggested to test the presence of PNH-type cells.
PlOl A case of nodular lymfoid M.J. Morgado, J. Fernandes, (Lisboa, P)
hyperplasia of the lung J. Cruz, M. MAlmeida,
J.L.
Ducla-Soares
Nodular lymfoid hyperplasia is a rare entity of unknown aetiology. Differential diagnostic consideration includes malignant lymphom of the lung. This is a controversial and probably benign entity, with a good prognosis after a surgical excision. The authors present a 40-year-old man, without clinical complaints until March 2002, when he began complaint of cough and six months later hemoptysis. The chest CT scan revealed a pulmonary nodule (4 X 3 cm) localised in the upper segment of
Medicine
14 (2003)
S61
SILT159
the left lower lobe, percutane biopsy was inconclusive: low-grade B cell lymphom (MALT type) versus inflammatory reaction with fibrosis and lipid pneumonia. A Gallium scintigraphy revealed a left pulmonary nodule, mediastinal and hilar lymph nodes hypercaptation. Autoimmune, immunodeficiency and viral diseases were excluded as well as linfoproliferative diseases. A lobectomy was performed in December 2002 and pathological examination revealed nodular lymphoid hyperplasia, and hilar lymph node inflammatory reaction. In reference to this case, considerations regarding clinical features, diagnosis, treatment and prognosis are made
P102 Lung involvement by non-Hodgkin (ALCL) A.R. MaJheiro, M.T. Cardoso, M. Chaves (Porte, P)
Anaplastic Sousa,
Large
F. Principe,
Cell Lymphoma R. Sousa,
F.C.
Anaplastic large-cell lymphoma (ALCL) represents a distinct group of lymphomas characterized by the expression of CD30 receptor. Primary systemic ALCL ALK+ is the most frequent sub-form accounting for 2 to 8% of all adult non-Hodgkin lymphomas, being commonly diagnosed in advanced stages, frequently with extra-nodal disease in two or more locations: skin (21%); bone (17%); soft tissue (17%); lung (11%); liver (8%); usually associated to systemic symptoms and fever. The authors describe the case of a 15 year old boy affected by ALCL, CD 30+, ALK+, T lineage, ‘small cell variant’, stage IV, with extra-nodal involvement of skin and medulla asymptomatic after first cycle of chemotherapy; 14 days after this cycle he developed fever, cough and dyspnea, as well as bilateral, peri-hilar, interstitial infiltration on chest X-ray. After 8 days of multiple antibiotherapies with no clinical response and several cultural studies performed including blood and sputum cultures as well as brochofibroscopy and bronchoalveolar lavage, it became apparent that this case probably corresponded to lung involvement by the lymphoma. The immediate day after stopping antibiotic and beginning a new chemotherapy cycle the patient became apyretic and in a few days became asymptomatic with total regression of the chest X-ray infiltration. When bronchovascular chest infiltrate is seen in a T-lineage non-Hodgkin lymphoma patient lung involvement by the lymphoma must always be suspected. Clinical manifestations are often nonspecific including fever, weight loss, generalized lymphadenopathy, dyspnea, chest pain, cough and hemoptisis and diagnosis is usually obtained by transbronchial or transthoracic biopsy or by needle aspiration of tissue or pleural fluid when there is pleural effusion which is not uncommon. Diagnosis is many times confirmed on the basis of regression or progression of disease with appropriate therapy. Lung lymphomatous involvement is often a difficult clinical and radiological diagnosis to make, yet it is many times critical in determining treatment.
P103 Primary Central Nervous System Lymphoma (PCNSL) with ocular involvement in non-hnmunocompromised patient. Relapse and treatment M.T. Cardoso, A. Malheiro, F. Principe, M. Damasceno, C. Chaves (Porto,P) PCNSL formerly rare is being seen with increased frequency among immunocompetent patients. Rare forms include oculocerebral lymphomas with poor prognosis. We report a case of a 42-year-old man who developed right frontotemporal headache, right visual acuity reduction and sixth nerve paresis, five years ago. A right cavernous sinus tumor was diagnosed and disappeared after partial resection and steroid therapy. Although he was clinically diagnosed to have a meningioma, the histologic examination verified infiltration of T and B small lymphoid
/ European
Journal
of Internal
disorders. Its occurs most frequently in elderly patients. Anti FVIII inhibitors have been reported in autoimmune diseases, lymphoproliferative disorders, cancers, post-partum, drug reactions and after transfusions. No predisposing factor was found in the reported cases, same as for 30% of patients. Bleeding is often severe, occuring spontaneously or after minor trauma. Overall mortality is 20%. Minor bleeding may require only local treatment or desmopressin. Steroids alone are useful in 30% of cases. Recombinant activated FVII or ACPP directly active the factor X. Serious bleeding may need high doses of FVIII. Other immunosuppressive drugs, plasmapheresis, intravenous immunoglobulines and rituximab have also been used. There is no consensus on the best therapeutic approach but it is always an expensive therapy. The two reported cases are particular because of the very old age of the patients. Mortality is probably high in this age group but there is no study on acquired haemophilia in very old patients.
Ploo Paroxysmal nocturnal hemoglohinuria and myelodysplasia A. Ruellan, T. Kestler, B. List, S. Scheringer, 0. Mainz, B. Schoenberger, F. Paul, M. Burkert (Dobel, Ingolstadt, D) Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired stem cell disorder due to a mutation of the X-linked phosphatidylinositol glycan complementation class A (PIG-A) gene. Mutated clones expresse a severe deficiency in all surface glycosylphosphatidylinositol (GPl)-anchored proteins (decay-accelerating factor=CD 55, membrane inhibitor of reactive lysis=CD 59, CB-binding protein=C8 bp), leading to an increased complement-mediated lysis. This disease presents in most of cases with episodes of hemoglobinuria and life-threatening venous thromboses. Its is associated with an aplastic anaemia (AA) in 30% of cases. Other haematological disorders such as myelodysplasia may be found but more rarely. We report a case of PNH with a myelodysplasia in a 37 year patient. In 1997 a PNH was diagnosed with a megaloblastic anaemia and a moderate transfusion requirement. He had no other complications of the PNH. In April 2002 he was admitted because of tiredness, with a megaloblastic (131 ~3) anaemia (haemoglobin 7 g/dl) without hemolysis. Platelets and leucocytes were normal. The proteins CD 55 and CD 59 were negative on red cells. The bone marrow aspiration was compatible with an hypoplastic myelodysplasia (refractory anaemia subtype). The bone marrow caryotype was normal. His state improved with erythrocytes transfusion. The relationship between myelodysplasia and PNH is not clear. The development of PHN clones requires a mutation of haematopoietic stem cells and an hypoplastic bone marrow environment, such as in AA or myelodysplasia. It has also been reported that the presence of a minor population of PNH-type cells in myelodysplasia is associated with a better prognosis, probably because of immunological mechanisms. PNH cells may escape damage and apoptosis because they have lost most of their ability to produce GPI. In the diagnosis procedure of a bone marrow failure it is suggested to test the presence of PNH-type cells.
PlOl A case of nodular lymfoid M.J. Morgado, J. Fernandes, (Lisboa, P)
hyperplasia of the lung J. Cruz, M. MAlmeida,
J.L.
Ducla-Soares
Nodular lymfoid hyperplasia is a rare entity of unknown aetiology. Differential diagnostic consideration includes malignant lymphom of the lung. This is a controversial and probably benign entity, with a good prognosis after a surgical excision. The authors present a 40-year-old man, without clinical complaints until March 2002, when he began complaint of cough and six months later hemoptysis. The chest CT scan revealed a pulmonary nodule (4 X 3 cm) localised in the upper segment of
Medicine
14 (2003)
S61
SILT159
the left lower lobe, percutane biopsy was inconclusive: low-grade B cell lymphom (MALT type) versus inflammatory reaction with fibrosis and lipid pneumonia. A Gallium scintigraphy revealed a left pulmonary nodule, mediastinal and hilar lymph nodes hypercaptation. Autoimmune, immunodeficiency and viral diseases were excluded as well as linfoproliferative diseases. A lobectomy was performed in December 2002 and pathological examination revealed nodular lymphoid hyperplasia, and hilar lymph node inflammatory reaction. In reference to this case, considerations regarding clinical features, diagnosis, treatment and prognosis are made
P102 Lung involvement by non-Hodgkin (ALCL) A.R. MaJheiro, M.T. Cardoso, M. Chaves (Porte, P)
Anaplastic Sousa,
Large
F. Principe,
Cell Lymphoma R. Sousa,
F.C.
Anaplastic large-cell lymphoma (ALCL) represents a distinct group of lymphomas characterized by the expression of CD30 receptor. Primary systemic ALCL ALK+ is the most frequent sub-form accounting for 2 to 8% of all adult non-Hodgkin lymphomas, being commonly diagnosed in advanced stages, frequently with extra-nodal disease in two or more locations: skin (21%); bone (17%); soft tissue (17%); lung (11%); liver (8%); usually associated to systemic symptoms and fever. The authors describe the case of a 15 year old boy affected by ALCL, CD 30+, ALK+, T lineage, ‘small cell variant’, stage IV, with extra-nodal involvement of skin and medulla asymptomatic after first cycle of chemotherapy; 14 days after this cycle he developed fever, cough and dyspnea, as well as bilateral, peri-hilar, interstitial infiltration on chest X-ray. After 8 days of multiple antibiotherapies with no clinical response and several cultural studies performed including blood and sputum cultures as well as brochofibroscopy and bronchoalveolar lavage, it became apparent that this case probably corresponded to lung involvement by the lymphoma. The immediate day after stopping antibiotic and beginning a new chemotherapy cycle the patient became apyretic and in a few days became asymptomatic with total regression of the chest X-ray infiltration. When bronchovascular chest infiltrate is seen in a T-lineage non-Hodgkin lymphoma patient lung involvement by the lymphoma must always be suspected. Clinical manifestations are often nonspecific including fever, weight loss, generalized lymphadenopathy, dyspnea, chest pain, cough and hemoptisis and diagnosis is usually obtained by transbronchial or transthoracic biopsy or by needle aspiration of tissue or pleural fluid when there is pleural effusion which is not uncommon. Diagnosis is many times confirmed on the basis of regression or progression of disease with appropriate therapy. Lung lymphomatous involvement is often a difficult clinical and radiological diagnosis to make, yet it is many times critical in determining treatment.
P103 Primary Central Nervous System Lymphoma (PCNSL) with ocular involvement in non-hnmunocompromised patient. Relapse and treatment M.T. Cardoso, A. Malheiro, F. Principe, M. Damasceno, C. Chaves (Porto,P) PCNSL formerly rare is being seen with increased frequency among immunocompetent patients. Rare forms include oculocerebral lymphomas with poor prognosis. We report a case of a 42-year-old man who developed right frontotemporal headache, right visual acuity reduction and sixth nerve paresis, five years ago. A right cavernous sinus tumor was diagnosed and disappeared after partial resection and steroid therapy. Although he was clinically diagnosed to have a meningioma, the histologic examination verified infiltration of T and B small lymphoid