glucose-6-phosphate dehydrogenase ( g6pd or g6pdh ) deficiency was first identified by dr . ernest beutler in 1953 . it is an x - linked recessive hereditary disease characterized by abnormally low levels of g6pd , a metabolic enzyme involved in the pentose phosphate pathway , especially important in red blood cell metabolism . g6pd deficiency is a prime example of a hemolytic anemia ( ha ) due to interaction between an intracorpuscular and an extracorpuscular cause , because in the majority of cases hemolysis is triggered by an exogenous agent . thus , the acute ha of g6pd deficiency is largely preventable by avoiding exposure to triggering factors of previously screened subjects . g6pd deficiency is the most common human enzyme defect , yet , there has been no reported case of g6pd deficiency associated with pancytopenia and bone loss . a 34-year - old female patient prediagnosed with g6pd deficiency complaining of gingival swelling and bleeding since 9 years and progressive spacing between maxillary and mandibular anterior teeth reported to the outpatient department of periodontology of the college . history dates back to the year 2002 when the patient developed some bleeding complications during her pregnancy and was admitted in the hospital . during her stay in the hospital , the patient showed signs of petechiae and ecchymosis on the extremities . it was then that she was diagnosed with aplastic anemia / pancytopenia with hypocellular bone marrow and g6pd deficiency . her platelet count kept decreasing and at the time of delivery her platelet count was 5000 only . the patient was then hospitalized for another 6 months after delivery during which her physical condition and her platelet count improved . since then the patient experienced gingival swelling and bleeding and progressive spacing between her upper and lower anterior teeth . she visited several general dentists but because of her underlying medical problem she was refused treatment by some while some provided her oral prophylaxis on her written consent . she developed some improvement in the oral condition but finally she was referred to the department of periodontology of the college for expert opinion . intra - oral examination showed signs of gingival inflammation , the changes being more pronounced in the maxillary and mandibular anterior region . the gingiva was dark pink , edematous , and enlarged with rolled and rounded margins with less pronounced scalloping . also , gingival recession and clefting was present on the palatal aspects of maxillary anterior teeth [ figures 1 and 2 ] . preoperative photograph showing papillary swelling and inflammation preoperative palatal view of maxillary arch showing clefting and inflammation of marginal gingiva and petechiae on palatal surface radiographically , generalized bone loss was evident which was more pronounced in the maxillary and mandibular anterior region [ figure 3 ] . preoperative opg showing bone loss mainly in upper and lower anterior teeth clinically , deep periodontal pockets were present in the maxillary and mandibular anterior teeth on probing using a periodontal probe with willams markings . g6pd deficiency , resulting from mutations in the g6pd gene , is well known in humans as a common cause of pathology , specifically of ha . all mutations that cause g6pd deficiency are found on the long arm of the x chromosome , on band xq28 . in many of these mutants , g6pd deficiency is not extreme and the clinical manifestations are limited , as they occur only when an exogenous agent triggers hemolysis . the clinical picture can develop upon ingestion of fava beans ( favism ) , during the course of infection , or after exposure to certain oxidative agents . g6pd - deficient red blood cells ( rbcs ) are more susceptible to oxidative stress and have a shorter lifespan . if a considerable proportion of hemolysis takes place in the spleen , as is often the case , splenomegaly may become a prominent feature and hypersplenism may develop , with consequent neutropenia and/or thrombocytopenia resulting in pancytopenia . oral changes include pale discoloration of the oral mucosa , increased susceptibility to infections , and bleeding spontaneously or on slightest provocation . gingival tissues represent an abnormal response to local irritation , the severity of which is dramatically alleviated by the removal of local irritant . studies have shown that accumulation of microbial biofilm , dental plaque , on tooth surfaces results in the development of an inflammatory process around gingival and periodontal tissues leading to their breakdown . also , increase in number of certain anaerobic bacteria in subgingival plaque due to hormonal changes occurs in pregnancy . moreover , maternal immune system is suppressed during pregnancy . thus , pregnancy and pancytopenia followed by hospitalization deteriorated the oral tissues of the patient . lack of proper dental treatment by the general dentists due to the patient 's underlying medical condition for about 9 years further exacerbated the oral problem of the patient to the present status . the treatment in the present scenario included proper oral prophylaxis , soft tissue debridement and surgical therapy after consultation and clearance from the hematologist and written consent from the patient . special care was taken regarding her current blood profile including all the relevant blood tests and individual cell counts , which were discussed in detail with her hematologist . all the drugs that are contraindicated in g6pd - deficient patients were avoided [ table 1 ] and only those drugs which are safe in g6pd - deficient patients were given [ table 2 ] . drugs and chemicals that should be avoided by persons with g6pd deficiency some common drugs that can be safely administered in therapeutic doses to glucose-6-phosphate dehydrogenase - deficient subjects without nonspherocytic hemolytic anemia the surgical therapy included open flap debridement and regenerative periodontal therapy wherever required and possible . the postoperative presentation included resolution of the patient 's symptoms of gingival bleeding and swelling . the clinical presentation shows reduction in the signs of gingival swelling , with reduction in size of tissues , reduction in probing depths and a significant gain in clinical attachment levels . there was no bleeding on probing and the signs of gingival inflammation were also resolved [ figures 46 ] .