Síndrome Hemofagocítico

24
A PROPOSITO DE UN CASO Ernesto García Ureta http://www.garciaureta.com/ XXIV Congreso de la SEAP

Transcript of Síndrome Hemofagocítico

Page 1: Síndrome Hemofagocítico

A PROPOSITO DE UN CASO

Ernesto Garciacutea Ureta

httpwwwgarciauretacomXXIV Congreso de la SEAP

Varoacuten de 11 antildeos

Linfoma no Hodgkin de alto grado de celulas Brico en celulas T

Asociado al virus de Epstein Barr

Diagnostico de Proceso Hemofagociticoen biopsia de Medula Osea

Ascitis

CD-68

S-100

1979 Sep44(3)993-1002

AbstractNineteen patients whose bone marrow smears showed histiocytic hyperplasia with prominent hemophagocytosis were found to have a clinicopathologic syndrome associated with active viral infection High fever constitutional symptoms liver function and coagulation abnormalities and peripheral blood cytopenias were characteristic findings Hepatosplenomegaly lymphadenopathy bilateral pulmonary infiltrates and skin rash were often present Fourteen of the patients were immunosuppressed Active infection by herpes group viruses was documented in 14 patients and by adenovirus in 1 The bone marrow of most patients also showed decreased granulopoiesis and erythropoiesis with normal to increased numbers of megakaryocytes Treatment generally consisted of supportive therapy and withdrawal of immunosuppressive drugs Thirteen patients recovered Lymph node biopsy and autopsy material showed generalized histiocytichyperplasia with hemophagocytosis The relationship of this disorder to familial hemophagocytic reticulosis familial erythrophagocytic lymphohistiocytosis histiocyticmedullary reticulosis and malignant histiocytosis is discussed Immunosuppressive and cytotoxic therapy may be contraindicated in the treatment of this virus-associated syndrome

Kimura S

Hemophagocytic Symdrome

Intern Med 205 44 278- 280

FIN

httpwwwgarciauretacom

Page 2: Síndrome Hemofagocítico

Varoacuten de 11 antildeos

Linfoma no Hodgkin de alto grado de celulas Brico en celulas T

Asociado al virus de Epstein Barr

Diagnostico de Proceso Hemofagociticoen biopsia de Medula Osea

Ascitis

CD-68

S-100

1979 Sep44(3)993-1002

AbstractNineteen patients whose bone marrow smears showed histiocytic hyperplasia with prominent hemophagocytosis were found to have a clinicopathologic syndrome associated with active viral infection High fever constitutional symptoms liver function and coagulation abnormalities and peripheral blood cytopenias were characteristic findings Hepatosplenomegaly lymphadenopathy bilateral pulmonary infiltrates and skin rash were often present Fourteen of the patients were immunosuppressed Active infection by herpes group viruses was documented in 14 patients and by adenovirus in 1 The bone marrow of most patients also showed decreased granulopoiesis and erythropoiesis with normal to increased numbers of megakaryocytes Treatment generally consisted of supportive therapy and withdrawal of immunosuppressive drugs Thirteen patients recovered Lymph node biopsy and autopsy material showed generalized histiocytichyperplasia with hemophagocytosis The relationship of this disorder to familial hemophagocytic reticulosis familial erythrophagocytic lymphohistiocytosis histiocyticmedullary reticulosis and malignant histiocytosis is discussed Immunosuppressive and cytotoxic therapy may be contraindicated in the treatment of this virus-associated syndrome

Kimura S

Hemophagocytic Symdrome

Intern Med 205 44 278- 280

FIN

httpwwwgarciauretacom

Page 3: Síndrome Hemofagocítico

CD-68

S-100

1979 Sep44(3)993-1002

AbstractNineteen patients whose bone marrow smears showed histiocytic hyperplasia with prominent hemophagocytosis were found to have a clinicopathologic syndrome associated with active viral infection High fever constitutional symptoms liver function and coagulation abnormalities and peripheral blood cytopenias were characteristic findings Hepatosplenomegaly lymphadenopathy bilateral pulmonary infiltrates and skin rash were often present Fourteen of the patients were immunosuppressed Active infection by herpes group viruses was documented in 14 patients and by adenovirus in 1 The bone marrow of most patients also showed decreased granulopoiesis and erythropoiesis with normal to increased numbers of megakaryocytes Treatment generally consisted of supportive therapy and withdrawal of immunosuppressive drugs Thirteen patients recovered Lymph node biopsy and autopsy material showed generalized histiocytichyperplasia with hemophagocytosis The relationship of this disorder to familial hemophagocytic reticulosis familial erythrophagocytic lymphohistiocytosis histiocyticmedullary reticulosis and malignant histiocytosis is discussed Immunosuppressive and cytotoxic therapy may be contraindicated in the treatment of this virus-associated syndrome

Kimura S

Hemophagocytic Symdrome

Intern Med 205 44 278- 280

FIN

httpwwwgarciauretacom

Page 4: Síndrome Hemofagocítico

S-100

1979 Sep44(3)993-1002

AbstractNineteen patients whose bone marrow smears showed histiocytic hyperplasia with prominent hemophagocytosis were found to have a clinicopathologic syndrome associated with active viral infection High fever constitutional symptoms liver function and coagulation abnormalities and peripheral blood cytopenias were characteristic findings Hepatosplenomegaly lymphadenopathy bilateral pulmonary infiltrates and skin rash were often present Fourteen of the patients were immunosuppressed Active infection by herpes group viruses was documented in 14 patients and by adenovirus in 1 The bone marrow of most patients also showed decreased granulopoiesis and erythropoiesis with normal to increased numbers of megakaryocytes Treatment generally consisted of supportive therapy and withdrawal of immunosuppressive drugs Thirteen patients recovered Lymph node biopsy and autopsy material showed generalized histiocytichyperplasia with hemophagocytosis The relationship of this disorder to familial hemophagocytic reticulosis familial erythrophagocytic lymphohistiocytosis histiocyticmedullary reticulosis and malignant histiocytosis is discussed Immunosuppressive and cytotoxic therapy may be contraindicated in the treatment of this virus-associated syndrome

Kimura S

Hemophagocytic Symdrome

Intern Med 205 44 278- 280

FIN

httpwwwgarciauretacom

Page 5: Síndrome Hemofagocítico

1979 Sep44(3)993-1002

AbstractNineteen patients whose bone marrow smears showed histiocytic hyperplasia with prominent hemophagocytosis were found to have a clinicopathologic syndrome associated with active viral infection High fever constitutional symptoms liver function and coagulation abnormalities and peripheral blood cytopenias were characteristic findings Hepatosplenomegaly lymphadenopathy bilateral pulmonary infiltrates and skin rash were often present Fourteen of the patients were immunosuppressed Active infection by herpes group viruses was documented in 14 patients and by adenovirus in 1 The bone marrow of most patients also showed decreased granulopoiesis and erythropoiesis with normal to increased numbers of megakaryocytes Treatment generally consisted of supportive therapy and withdrawal of immunosuppressive drugs Thirteen patients recovered Lymph node biopsy and autopsy material showed generalized histiocytichyperplasia with hemophagocytosis The relationship of this disorder to familial hemophagocytic reticulosis familial erythrophagocytic lymphohistiocytosis histiocyticmedullary reticulosis and malignant histiocytosis is discussed Immunosuppressive and cytotoxic therapy may be contraindicated in the treatment of this virus-associated syndrome

Kimura S

Hemophagocytic Symdrome

Intern Med 205 44 278- 280

FIN

httpwwwgarciauretacom

Page 6: Síndrome Hemofagocítico

Kimura S

Hemophagocytic Symdrome

Intern Med 205 44 278- 280

FIN

httpwwwgarciauretacom

Page 7: Síndrome Hemofagocítico

FIN

httpwwwgarciauretacom