Hematological response of pancytopenia to glucocorticoids in patients with Sheehan's syndrome.

Sheehan's syndrome presents with panhypopituitarism after childbirth, usually preceded by post partum hemorrhage. Hematological abnormalities like pancytopenia with hypocellular marrow in these patients are reported rarely. Though multiple hormone deficiencies may contribute to Pancytopenia in Sheehan's syndrome, complete recovery is observed after achieving eucortisolemic and euthyroid state. The predominant role of thyroxine or glucocorticoids in reversing pancytopenia in these patients has not been studied. We present the clinical, hormonal, hematological course and response to glucocorticoids in a patient of Sheehan's syndrome presenting with pancytopenia. Complete recovery of pancytopenia was observed after achieving eucortisolemic state thus concluding that gulcocorticoid replacement is sufficient to reverse pancytopenia in these patients.

Relation between IRF-1 gene and acute myelocytic leukemia in Kashmiri population.

The IRF-1 protein, a mammalian transcriptional factor encoded by a gene located in 5q23-q31, has antioncogenic properties. Involved in regulation of differentiation and proliferation, IRF-1 acts as a tumor suppressor gene and is inactivated by deletion of its one or more exons (exon skipping) in many hematological malignancies, including acute myelocytic leukemia (AML) and myelodysplastic syndromes (MDS). DNA samples, extracted from peripheral blood, taken from 50 Kashmiri AML subjects, were analysed using the polymerase chain reaction and compared with examples of age and gender matched healthy controls from the same population. Three different exon regions (2, 3 and 4) of the IRF-1 gene that were previously shown to be prone to deletion were selected for amplification and analysis. Deletion was observed in 31(62%) out of 50 AML patients (p=0.016). Exon 3 was most frequently deleted (58%), followed by exon 2 (28%), while exon 4 was least affected (12%), providing insights into critical roles in leukemogenesis. The number of deleted exons was variable, but single exon deletions were more frequent (30%). Of interest, IRF-1 gene deletions were not observed in 19 (38%) patients. In our study, the frequency of deletions of these three exons was slightly higher than in an Indian population (52%), but lower than in Sweden in Europe (95%). This study also explored the prevalence and clinical profile of IRF-1 deletions in AML patients. Adults had a significantly higher incidence than children (p=0.0168) and IRF-1 deletions were associated with low Hb (p< 0.0001), high TLC (p=0.0033) and a low platelet count (p=0.0076).

Prevalence of hematological abnormalities in patients with Sheehan's syndrome: response to replacement of glucocorticoids and thyroxine.

Anemia and other hematological abnormalities are common in patients with Sheehan's syndrome. The response of these abnormalities to replacement of thyroxine and glucocorticoids is not clear. The aim of the present study was to document the profile of hematological abnormalities and response to treatment in patients with Sheehan's syndrome. Forty patients of Sheehan's syndrome and an equal number of age and parity matched healthy controls were studied for prevalence of hematological abnormalities. Hemoglobin concentration, hematocrit, red cell, white cell and platelet count were significantly decreased in patients with Sheehan's syndrome compared to controls. Frequency of anemia, leucopenia, thrombocytopenia and pancytopenia was significantly higher in these patients compared to controls. After achieving euthyroid and eucortisol state, there was a complete recovery of these hematological abnormalities. We conclude that anemia and other cytopenias are common in patients with Sheehan's syndrome and replacement with thyroxine and glucocorticoids results in complete recovery of these abnormalities.