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INDIAN JOURNAL OF MEDICAL SCIENCES 63 64 the lymphocyte yield is suboptimal due to 4. Farrell K, Connolly MB, Munn R, Peng S, PAROXYSMAL NOCTURNAL HEMOGLOBINURIA IN CHILDHOOD: death of lymphocytes in vivo and this can MacWilliam LM. Prospective, open-label, add-on AN UNCOMMON PRESENTATION lead to increased chances of a false-negative study of lamotrigine in 56 children with intractable result.[7] Also, the test is conducted in few generalized epilepsy. Pediatr Neurol 1997;16:201- V. GUPTA, V. TILAK*, S. V. K. RAMAKRISHNA, B. D. BHATIA research laboratories which limits its 5. 5. Thome-Souza S, Freitas A, Fiore LA, Valente KD. accessibility to physicians.[7] Lamotrigine and valproate: efficacy of co- ABSTRACT administration in a pediatric population. Pediatr To conclude, timely recognition of AHS, a Neurol 2003;28:360-4. An eight year old boy presented with severe and spots. Complete rare but potentially fatal drug reaction is 6. Shear NH, Spielberg SP. Anticonvulsant blood count showed . There was mild reticulocytosis. was important. Discontinuation of the offending hypersensitivity syndrome. In vitro assessment hypocellular with normoblastic erythroid hyperplasia. Ham’s test (acidified serum test) anticonvulsant and supportive, symptomatic of risk. J Clin Invest 1988;82:1826-32. was positive which confirmed the diagnosis of Paroxysmal nocturnal hemoglobinuria therapy forms the mainstay of 7. Gogtay NJ, Bavdekar SB, Kshirsagar NA. (PNH). Although PNH is rare in childhood, it should be considered as a diagnostic [7] management. Use of corticosteroids and Anticonvulsant hypersensitivity syndrome: a possibility in cases of as the two conditions can coexist. The presence intravenous immunoglobulin in severe cases review. Expert Opin Drug Saf 2005;4:571-81. of PNH in association with aplastic anemia can influence the outcome of the latter. has been suggested.[7] Once the patient 8. Naranjo CA, Busto U, Sellers EM, Sandor P, Ruiz recovers from the acute episode, another I, Roberts EA, et al. A method for estimating the Key words: Paroxysmal nocturnal hemoglobinuria, aplastic anemia, pancytopenia structurally dissimilar anticonvulsant should probability of adverse drug reactions. Clin Pharmacol Ther 1981;30:239-45. be started. Options available include valproic 9. Schlienger RG, Knowles SR, Shear NH. acid, clobazam, gabapentin, vigabatrin and Lamotrigine-associated anticonvulsant topiramate.[7] INTRODUCTION of PNH type cells in cases of bone marrow hypersensitivity syndrome. Neurology failure syndromes suggests a relatively 1998;51:1172-5. Paroxysmal nocturnal hemoglobinuria (PNH) benign nature of the disease.[2] An attempt REFERENCES 10. Chattergoon DS, McGuigan MA, Koren G, Hwang P, Ito S. Multiorgan dysfunction and disseminated is an acquired clonal hematologic disorder should be made to identify the condition 1. Alldredge BK, Knutsen AP, Ferriero D. Antiepileptic intravascular in children receiving which has been traditionally classified as before starting therapy. Majority of the cases drug hypersensitivity syndrome: in vitro and clinical lamotrigine and valproic acid. Neurology . However, it is also occur in adulthood with isolated case reports observations. Pediatr Neurol 1994;10:169-71. 1997;49:1442-4. associated with a component of bone marrow in children.[3,4] An extensive literature search 2. Vittorio CC, Muglia JJ. Anticonvulsant 11. Brown TS, Appel JE, Kasteler JS, Callen JP. failure and a liability for venous thrombosis. revealed only one case report from India in hypersensitivity syndrome. Arch Intern Med Hypersensitivity reaction in a child due to It is a unique disorder characterized by a a child[5] which prompted us to report an 1995;155:2285-90. lamotrigine. Pediatr Dermatol 1999;16:46-9. triad of hemolytic anemia, pancytopenia and interesting case of severe anemia in an eight 3. Bessmertny O, Hatton RC, Gonzalez-Peralta RP. 12. Schaub N, Bircher AJ. Severe hypersensitivity thrombosis.[1] There can be either sequential year old boy which turned out to be PNH with Antiepileptic hypersensitivity syndrome in children. syndrome to lamotrigine confirmed by lymphocyte or concurrent appearance of aplastic anemia aplastic anemia. stimulation in vitro. Allergy 2000;55:191-3. Ann Pharmacother 2001;35:533-8. and PNH in an individual patient. Presence CASE REPORT

Departments of Pediatrics and *Pathology Institute of Medical Sciences, Banaras Hindu University, An eight year old boy presented to Pediatric Varanasi – 221 005, India out-patient department in January 2004 with the complaints of progressively increasing Correspondence Vineeta Gupta and fatigability for 2 months and Department of Pediatrics, Institute of Medical Sciences, bleeding spots all over the body for 1 month B.H.U, Varanasi 221 005, India E-mail: [email protected] duration. On, examination, the child had

Indian J Med Sci, Vol. 60, No. 2, February 2006 Indian J Med Sci, Vol. 60, No. 2, February 2006 INDIAN JOURNAL OF MEDICAL SCIENCES 65 66 PAROXYSMAL NOCTURNAL HEMOGLOBINURIA severe pallor with petechial and purpuric DISCUSSION another. The cause of bone marrow failure in Treatment of the condition depends upon the spots all over the body. The liver was PNH is not very clear. The possible clinical presentation. In patients presenting palpable 2 cm below the costal margin and PNH was first described a century ago but explanations are that PNH clone suppresses with the features of aplastic anemia, either spleen was 1 cm. There was no significant the molecular defects have been identified the normal marrow progenitors or it has an cyclosporine (Cy A) alone or in combination lymphadenopathy. On investigation, the only recently. Because of a somatic mutation intrinsic growth and proliferative advantage with antithymocyte globulin (ATG) have been hemoglobin was 6.2 gm%, total leucocyte in the phosphatidyl inositol glycan compared to normal stem cells.[1] The high tried. Results of a combination treatment count was 3550/mm3 with a differential count complementation group-A (PIG-A) gene, a prevalence of bone marrow failure frequently (ATG + Cy A) are better than Cyclosporine of 38% neutrophils, 2% eosinophils and 60% number of proteins such as membrane leads to an initial diagnosis of aplastic alone.[8,9] Allogenic haemopoietic cell lymphocytes. The count was 18000/ inhibitor of reactive lysis (MIRL, CD59) and anemia rather than PNH as happened in the transplantation has been tried successfully.[10] mm3. There were no abnormal cells in the decay accelerating factor (DAF, CD55) are present case. He was initially diagnosed as In the present case cyclosporine was used peripheral smear. Bone marrow aspiration absent from the surface of red blood cells.[1] a case of aplastic anemia but because of the alone because of financial constraints. In was hypocellular with normoblastic erythroid This renders the cells susceptible to lysis by presence of normoblastic erythroid cases with recurrent hemolytic episodes short hyperplasia. The trephine biopsy showed complement resulting in intravascular hyperplasia in the bone marrow and mild courses of oral prednisolone have been tried decreased cellularity. The leucopoeisis and hemolysis and hemoglobinuria. The reticulocytosis, the possibility of PNH was around the time of hemolysis.[1] thrombopoeisis were depressed with a hemoglobinuria may be intermittent on thought of. The reticulocyte count in these myeloid to erythroid ratio of 1.5:1. The awakening in the morning or may be cases is not parallel with the extent of REFERENCES reticulocyte count was 2.5% with a negative continuous throughout the day. However, hemolysis and this reflects the underlying Coomb’s test. The serum LDH was 700 IU/ some patients may never develop it. bone marrow failure which always coexists 1. Luzzatto L, Notaro R. Paroxysmal nocturnal L. Serum bilirubin was within normal limits with PNH.[1] Venous thrombosis is the third hemoglobinuria. In: Handin RI, Lux SE, Stossel TP and urine examination was negative for PNH is a disease of adults presenting in 3rd component of this condition, the editors. Blood: Principles and Practice of hemoglobinuria. In view of the normoblastic to 5th decade. In a large series of 78 cases pathophysiology of which is not very clear. . Philadelphia; Lippincott Williams & Wilkins: 2003. p. 318-32. erythroid hyperplasia in the bone marrow and of PNH diagnosed in 10 years, the mean The possible reasons are impaired 2. Wang H, Chuhjo T, Yasue S, Omine M, Nakao S. reticulocytosis against a backdrop of age of diagnosis was 34 years.[6] In the fibrinolysis, hypercoagulability and Clinical significance of a minor population of PNH ­ pancytopenia, a diagnosis of Paroxysmal largest series of 26 cases of PNH with onset hyperactivity of because of the lack type cells in bone marrow failure syndrome. Blood nocturnal hemoglobinuria was considered. in childhood the age ranged from 8 months of CD59 on PNH platelets. However, a 2002;100:3897-902. Ham’s test (acidified serum test) was carried to 21 years and only 3.8% cases were thrombotic complication was not present in 3. Dolzel Z, Dostalkova D, Blatny J, Starha J, [7] out which was positive. The final diagnosis younger than 10 years. There was our case. Gerycova H. Paroxysmal nocturnal was PNH with aplastic anemia. The child was significant difference between young hemoglobinuria in a girl with hemolysis and given supportive treatment with packed red patients with PNH and adult patients. Ham’s test is a highly sensitive test for the “hematuria”. Pediatr Nephrol 2004;19:1177-9. cells and platelet transfusion and was started Hemoglobinuria as the presenting complaint diagnosis of PNH. It may miss the diagnosis 4. Wainright L, Brodsky RA, Eranus LK, Poyiadjis S, on oral cyclosporine A alone as he could not was seen in only 15% of children as against initially if the PNH clone is small. Therefore Naidu G, Mckinnon D. Paroxysmal nocturnal afford treatment with antithymocyte globulin. 50% in adults. In contrast, bone marrow it is important to repeat the Ham’s test at hemoglobinuria arising from Fanconi anemia. J Two months in follow up, the child continued failure was much more common in young regular intervals to avoid missing the Pediatr Hematol Oncol 2003;25:167-8. 5. Yaranal PJ, Basu D, Narayanan P, Mahadevan S. to have anemia and pancytopenia but there than in the adults (58% versus 25%). diagnosis. The availability of flow-cytometry Paroxysmal nocturnal hemoglobinuria with onset was some improvement in the has further improved the diagnosis of PNH. in childhood: a case report. Indian J Pathol Microbiol . The platelet count rose to PNH and aplastic anemia are clinically In the series of 78 cases of PNH by Zhao et 2004;47:233-5. 46,000/mm3. Unfortunately, the child was lost related. The disorders may present al[6] Ham’s test was positive in 65.8% cases 6. Zhao M, Shao Z, Li K, Chen G, Liu H, Zhang Y. to follow up subsequently. simultaneously or one may evolve into whereas CD59 and CD55 were found Clinical analysis of 78 cases of paroxysmal deficient in 100% of cases. nocturnal hemoglobinuria diagnosed in past 10

Indian J Med Sci, Vol. 60, No. 2, February 2006 Indian J Med Sci, Vol. 60, No. 2, February 2006 INDIAN JOURNAL OF MEDICAL SCIENCES 67 68

years. Chin Med J 2002;115:398-402. complement activation and transient decrease of PNH LETTER TO THE EDITOR 7. Ware RE, Hall SE, Rosse WF. Paroxysmal clone. Immunobiology 1996;196:513-21. nocturnal hemoglobinuria with onset in childhood 9. Saso R, Marsh J, Cevreska L, Szer J, Gale RP, and adolescence. New Eng J Med 1991;325:991­ Rowlings PA, et al. Bone marrow transplantation for were isolated, identified as B. pseudomallei. 5. PNH. Br J Haematol 1999;104:392-6. MULTIPLE VISCERAL ABSCESS 8. Ebenbichler CF, Wurfzner R, Sandhofer AD, 10. Lee JL, Lee JH, Lee JH, Choi SJ, Kim S, Seol M. IN A CASE OF MELIOIDOSIS Two samples of blood culture were also Niederweiser D, Dierich MP, Patsch JR. Anti Allogenic hematopoetic cell transplantation for positive. The medical treatment was switched thymocyte globulin treatment of a patient for PNH paroxysmal nocturnal hemoglobinuria. Eur J Hematol to ceftazidime (2 g q 8h). The patient’s haemoglobinuria- aplastic anemia syndrome: 2003;71:114-8. Sir, clinical condition did not improve and the Melioidosis is an caused by patient succumbed to his illness on the Burkholderia pseudomallei, an important seventh day after admission. human pathogen in tropical areas, but rarely thought of and investigated in India, where sporadic cases are reported. We report an unusual case of melioidosis with multiple abscesses in the liver and spleen in a diabetic elderly man from India with no travel history who presented with prolonged fever. Diagnosis of melioidosis was from the blood and pus culture.

A 51-year old diabetic man from Orissa presented with a history of low-grade fever, progressive left-lower-quadrant abdominal Figure 1: CT scan of abdomen showing mild hepatomegaly with few small abscesses in both lobes pain for 3 months. Abdominal examination and also splenomegaly reveling multiple splenic revealed non-tender hepatosplenomegaly. abscess. Hematological and biochemical indices, apart from a haematocrit of 23.9%, erythrocyte sedimentation rate of 80 mm, alkaline phosphatase of 226 U/L (50-136) were within normal range. The chest radiograph was normal. Computerized tomography (CT) of the abdomen showed the presence of multiple hypo dense lesions in the liver and spleen [Figures 1 and 2]. The patient was initially put on ceftriaxone and metronidazole. The pus from the liver abscess was aspirated under ultrasound guidance and was cultured. Two Figure 2: CT scan of the abdomen showing days after incubation, scanty growths of splenomegaly with multiple splenic abscesses and a gram-negative non-lactose fermenter colonies septated perisplenic fluid collection.

Indian J Med Sci, Vol. 60, No. 2, February 2006 Indian J Med Sci, Vol. 60, No. 2, February 2006