Abdominal tuberculosis with autoimmune hemolytic anemia
http://www.100md.com
《美国医学杂志》
Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India
Abstract
An eight-year-old male child presented with progressive distension of abdomen, fever, pallor and jaundice with a history of tubercular contact. Investigations were suggestive of abdominal tuberculosis with autoimmune hemolytic anemia. The child responded well to a course of oral steroids with antitubercular treatment. A literature search did not reveal any previous case report of an association between tuberculosis and autoimmune hemolytic anemia.
Keywords: Autoimmune hemolytic anemia; Tuberculosis
Autoimmune hemolytic anemias are a group of disorders in which endogenously produced antibodies (autoantibodies) are directed against RBCs resulting in reduced life span of cells and clinical manifestations of pallor, jaundice and splenomegaly. [1] The condition is seen in both children and adults and even in infants.[2] This may be idiopathic or secondary to a number of underlying diseases and infections. The authors report a case of autoimmune hemolytic anemia in association with abdominal tuberculosis which to our knowledge has not been reported earlier.
Case report
An 8 year old boy presented with history of progressive abdominal distension associated with moderate grade intermittent fever for four months and pallor and jaundice for 15 days. There was a history of tubercular contact in the family. An uncle was suffering from pulmonary tuberculosis and receiving antitubercular treatment for the last 6 months. On examination, the child was thin built weighing 19.7 kg. There was moderate pallor and icterus. Abdomen was distended with mild hepatosplenomegaly and evidence of free fluid in the form of shifting dullness. There was no significant lymphadenopathy or pedal edema. Chest and cardiovascular system examination was not contributory.
Investigations revealed a hemoglobin of 8 gm% with total leucocyte count of 6000/mm[3] and normal differential leucocyte count. General blood picture revealed predominantly normochromic normocytic erythrocytes with normal platelets. The reticulocyte count was 4%. Total serum bilirubin was 2.5 mg% with 0.9 mg% direct and the rest indirect bilirubin. Total proteins, Albumin:Globulin ratio and liver enzymes (SGOT, SGPT) were within normal limits. Ultrasonography of the abdomen revealed multiple dilated bowel loops with small ascites. On barium meal follow through examination, multiple strictures and dilatations in the small intestine, suggestive of intestinal tuberculosis were seen. Mantoux test was positive. Six days after admission, hemoglobin of the child dropped to 6 gm% and serum bilirubin increased to 4.6 mg% with an indirect component of 3.0 mg%. At this stage, the general blood picture revealed anisopoikilocytosis with 6 % reticulocytosis. Bone marrow examination showed marked erythroid hyperplasia with normal myelopoiesis and megakaryocytes. A possibility of autoimmune hemolytic anemia was thought of and direct antiglobulin test (Coomb's test) was done which was positive. A repeat test after one week was also positive.
Based on the clinical features and investigations a final diagnosis of abdominal tuberculosis with autoimmune hemolytic anemia was reached. The child was put on antitubercular treatment with oral prednisolone. Prednisolone was gradually tapered off after 4 weeks and antitubercular treatment was continued. The child made an uneventful recovery and there was no exacerbation of anemia after stopping steroids. Six months later, the child is well in follow-up. His hemoglobin is 11 gm%.
Discussion
In the autoimmune hemolytic anemias, the autoantibody may be produced as an inappropriate immune response to a RBC antigen. Alternatively, an infectious agent may in some way alter the RBC membrane so that it becomes "foreign" or antigenic to the host.[3] The hallmark of this group of diseases is a positive direct antiglobulin (Coombs) test which was positive on two different occasions in this case. In many instances, no underlying cause can be found and it is then considered to be primary or idiopathic autoimmune hemolytic anemia (AIHA). It may be secondary to a number of underlying diseases like lymphoproliferative, immunologic and connective tissue disorders, neoplasms[4] and infections.[5] Multiple infectious agents like Epstein Barr virus, cytomegalovirus, HIV, mumps, measles, rubella, mycoplasma and rarely visceral leishmaniasis[6] have been described in association with secondary autoimmune hemolytic anemia. Even acute bacterial infections can lead to this condition. An unusual case of pulmonary caseating granuloma secondary to Mycoplasma pneumoniae nfection was described with autoimmune hemolytic anemia.[7] However, an extensive literature search failed to show an association of autoimmune hemolytic anemia with tubercular infection and therefore we thought of reporting this case.
The treatment of autoimmune hemolytic anemia is either oral prednisolone or its equivalent. The dose is gradually reduced and if relapse occurs, resumption of full dosage may be necessary. In resistant cases, intravenous immunoglobulins[8] or immunosuppressive agents have been tried. In the present case, the patient responded to a course of oral prednisolone and is doing well in follow-up.
References
1. Segel GB: Hemolytic anemias resulting from extracellular factors. In Behrman RE, Kleigman RM, Jenson HB, eds. Nelson Textbook of Pediatrics, 17th edn. Saunders, Philadelphia, 2004; 1638-1640.
2. Choudhary VP, Passi GR, Pati HP. Clinico-haematological spectrum of autoimmune hemolytic anemia. An Indian experience. J Assoc Physicians India 1996; 44: 112-116.
3. Schreiber AD, Gill FM, Manno CS. Autoimmune hemolytic anemia. In: Nathan DG, Orkin SH eds. Nathan & Oski's Hematology of Infancy and Childhood. 5th edn. Philadelphia, W.B. Saunders Company, 1998; 1: 501-506.
4. Kiran PS, Sowdi VP, Shetty SB, Venugopal V. Sinus histiocytosis with massive lymphadenopathy: complicated by autoimmune hemolytic anemia. Indian J Pediatr 2003; 70: 759-760.
5. Agarwal B. Autoimmune hemolytic anemia. Indian J Pediatr 1998; 65: 663-668.
6. Adil SN, Burney IA, Khurshid M. Autoimmune hemolytic anemia in visceral leishmaniasis. J Pak Med Assoc 1999; 49: 100-101.
7. Linz D, Kessler S, Kane J. Pulmonary caseating granuloma associated with Mycoplasma pneumoniae pneumonia. South Med J 1983; 76: 1429-1432.
8. Choudhary VP, Mahapatra M, Kashyap R. Immunoglobulin therapy in immunohemolytic disorder. Indian J Pediatr 1998; 65: 681-690.(Gupta V, Bhatia BD)
Abstract
An eight-year-old male child presented with progressive distension of abdomen, fever, pallor and jaundice with a history of tubercular contact. Investigations were suggestive of abdominal tuberculosis with autoimmune hemolytic anemia. The child responded well to a course of oral steroids with antitubercular treatment. A literature search did not reveal any previous case report of an association between tuberculosis and autoimmune hemolytic anemia.
Keywords: Autoimmune hemolytic anemia; Tuberculosis
Autoimmune hemolytic anemias are a group of disorders in which endogenously produced antibodies (autoantibodies) are directed against RBCs resulting in reduced life span of cells and clinical manifestations of pallor, jaundice and splenomegaly. [1] The condition is seen in both children and adults and even in infants.[2] This may be idiopathic or secondary to a number of underlying diseases and infections. The authors report a case of autoimmune hemolytic anemia in association with abdominal tuberculosis which to our knowledge has not been reported earlier.
Case report
An 8 year old boy presented with history of progressive abdominal distension associated with moderate grade intermittent fever for four months and pallor and jaundice for 15 days. There was a history of tubercular contact in the family. An uncle was suffering from pulmonary tuberculosis and receiving antitubercular treatment for the last 6 months. On examination, the child was thin built weighing 19.7 kg. There was moderate pallor and icterus. Abdomen was distended with mild hepatosplenomegaly and evidence of free fluid in the form of shifting dullness. There was no significant lymphadenopathy or pedal edema. Chest and cardiovascular system examination was not contributory.
Investigations revealed a hemoglobin of 8 gm% with total leucocyte count of 6000/mm[3] and normal differential leucocyte count. General blood picture revealed predominantly normochromic normocytic erythrocytes with normal platelets. The reticulocyte count was 4%. Total serum bilirubin was 2.5 mg% with 0.9 mg% direct and the rest indirect bilirubin. Total proteins, Albumin:Globulin ratio and liver enzymes (SGOT, SGPT) were within normal limits. Ultrasonography of the abdomen revealed multiple dilated bowel loops with small ascites. On barium meal follow through examination, multiple strictures and dilatations in the small intestine, suggestive of intestinal tuberculosis were seen. Mantoux test was positive. Six days after admission, hemoglobin of the child dropped to 6 gm% and serum bilirubin increased to 4.6 mg% with an indirect component of 3.0 mg%. At this stage, the general blood picture revealed anisopoikilocytosis with 6 % reticulocytosis. Bone marrow examination showed marked erythroid hyperplasia with normal myelopoiesis and megakaryocytes. A possibility of autoimmune hemolytic anemia was thought of and direct antiglobulin test (Coomb's test) was done which was positive. A repeat test after one week was also positive.
Based on the clinical features and investigations a final diagnosis of abdominal tuberculosis with autoimmune hemolytic anemia was reached. The child was put on antitubercular treatment with oral prednisolone. Prednisolone was gradually tapered off after 4 weeks and antitubercular treatment was continued. The child made an uneventful recovery and there was no exacerbation of anemia after stopping steroids. Six months later, the child is well in follow-up. His hemoglobin is 11 gm%.
Discussion
In the autoimmune hemolytic anemias, the autoantibody may be produced as an inappropriate immune response to a RBC antigen. Alternatively, an infectious agent may in some way alter the RBC membrane so that it becomes "foreign" or antigenic to the host.[3] The hallmark of this group of diseases is a positive direct antiglobulin (Coombs) test which was positive on two different occasions in this case. In many instances, no underlying cause can be found and it is then considered to be primary or idiopathic autoimmune hemolytic anemia (AIHA). It may be secondary to a number of underlying diseases like lymphoproliferative, immunologic and connective tissue disorders, neoplasms[4] and infections.[5] Multiple infectious agents like Epstein Barr virus, cytomegalovirus, HIV, mumps, measles, rubella, mycoplasma and rarely visceral leishmaniasis[6] have been described in association with secondary autoimmune hemolytic anemia. Even acute bacterial infections can lead to this condition. An unusual case of pulmonary caseating granuloma secondary to Mycoplasma pneumoniae nfection was described with autoimmune hemolytic anemia.[7] However, an extensive literature search failed to show an association of autoimmune hemolytic anemia with tubercular infection and therefore we thought of reporting this case.
The treatment of autoimmune hemolytic anemia is either oral prednisolone or its equivalent. The dose is gradually reduced and if relapse occurs, resumption of full dosage may be necessary. In resistant cases, intravenous immunoglobulins[8] or immunosuppressive agents have been tried. In the present case, the patient responded to a course of oral prednisolone and is doing well in follow-up.
References
1. Segel GB: Hemolytic anemias resulting from extracellular factors. In Behrman RE, Kleigman RM, Jenson HB, eds. Nelson Textbook of Pediatrics, 17th edn. Saunders, Philadelphia, 2004; 1638-1640.
2. Choudhary VP, Passi GR, Pati HP. Clinico-haematological spectrum of autoimmune hemolytic anemia. An Indian experience. J Assoc Physicians India 1996; 44: 112-116.
3. Schreiber AD, Gill FM, Manno CS. Autoimmune hemolytic anemia. In: Nathan DG, Orkin SH eds. Nathan & Oski's Hematology of Infancy and Childhood. 5th edn. Philadelphia, W.B. Saunders Company, 1998; 1: 501-506.
4. Kiran PS, Sowdi VP, Shetty SB, Venugopal V. Sinus histiocytosis with massive lymphadenopathy: complicated by autoimmune hemolytic anemia. Indian J Pediatr 2003; 70: 759-760.
5. Agarwal B. Autoimmune hemolytic anemia. Indian J Pediatr 1998; 65: 663-668.
6. Adil SN, Burney IA, Khurshid M. Autoimmune hemolytic anemia in visceral leishmaniasis. J Pak Med Assoc 1999; 49: 100-101.
7. Linz D, Kessler S, Kane J. Pulmonary caseating granuloma associated with Mycoplasma pneumoniae pneumonia. South Med J 1983; 76: 1429-1432.
8. Choudhary VP, Mahapatra M, Kashyap R. Immunoglobulin therapy in immunohemolytic disorder. Indian J Pediatr 1998; 65: 681-690.(Gupta V, Bhatia BD)