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H P Pati

Publications and source records attributed to H P Pati.

At least 19 recordsLinked to original sources
PubMed2004

Sickle beta-thalassemia presenting as orbital compression syndrome.

Orbital compression syndrome is caused by disorders of varying etiologies involving the orbit and presents with ocular and extraocular dysfunction. Ocular involvement of sickle cell disease is uncommon. A 17-year-old male presented with low backache and proptosis of both eyes of 5 days duration without past history of pain crisis or transfusion. Examination revealed pallor, icterus, bilateral proptosis, conjuctival chemosis, and symmetrical restriction of ocular movements with preserved visual acuity. He was drowsy with no other focal deficits. The fundus showed macular edema, venous engorgement, and no hemorrhage. His peripheral smear showed presence of sickle cells. Computed tomography (CT) scan of the orbit revealed orbital subperiosteal hematomas. CT head images showed epidural hematoma in the frontal lobe. High-performance liquid chromatography (HPLC) and mutation studies revealed sickle beta-thalassemia in the patient. He was managed with supportive care, transfusions to keep hemoglobin above 10 g/dl, and hydroxyurea. The patient recovered fully and remained well during follow-up of 12 months. Our case was unique for presenting as orbital compression syndrome without any history of vaso-occlusive crisis.

Adolescent↗
PubMed2004

Deferiprone, efficacy and safety.

OBJECTIVE: Deferiprone (L1), the new oral iron chelator has been studied in several countries for its efficacy and toxicity with some conflicting observations. Toxicity involving joints has been reported more frequently in Indian patients. The authors planned to include larger number of Indian thalassemics in studying safety and efficacy of Deferiprone. METHODS: Seventy five thalassemic children (4-14 yr) were studied for one year with various investigations done periodically. Thirty patients (group A) received 50 mg/kg dose and 21 others (group B) received 75 mg/kg dose of Deferiprone. Rest of the patients were followed up without any chelator. RESULTS: The serum ferritin levels reduced significantly in both groups (P < 0.01 each); more in 75 mg/kg than the 50 mg/kg group. Arthropathy appeared in 15 (50%) patients in Group A and 6 (28.6%) of Group B after 1-12 (mean 6) months of L1 treatment; however, only one patient needed withdrawal of L1. Eleven patients needed indomethacin for pain relief. Seropositivity for antinuclear factor and rheumatoid factor had no relation to dose or duration of L1 therapy, arthropathy or the serum ferritin level. Twelve patients developed leucopenia (< 3.0 x 10(9)/L) and neutropenia (0-1.8 x 10(9)/L) after 2-11 months of L1 therapy and was not related to the dose or duration of therapy. The drug was restarted in 10 patients and only one of them developed a second episode of neutropenia. CONCLUSION: Deferiprone is an effective iron chelator, but arthropathy and neutropenia are very frequent side effects and need strict monitoring during therapy. Most of the neutropenia are neither very severe nor recur with re-challenge with the drug. Similarly, arthropathy does not need withdrawal of drug in majority of patients.

Adolescent↗
PubMed2004

Clinico-haematological profile of HbE syndrome in adults and children.

Haemoglobin E beta thalassemia (HbE beta thalassemia) has a remarkable variability in clinical expression ranging from a mild form of thalassemia intermedia to a transfusion dependent condition. An overlap between the mild variety of HbE beta thalassemia and homozygous HbE disease is common, however, differentiation is required for early institution of therapy and for predicting the later clinical course. Fifty cases of Hb E syndrome comprising of 43 cases of Hb E beta thalassemia and 7 cases of homozygous HbE disease were studied. Their clinico- haematological features and results of high performance liquid chromatography (HPLC) were analysed.

Adult↗
PubMed2004

Clinical, haematological and histomorphological profile of adult myelodysplastic syndrome. Study of 96 cases in a single institute.

Myelodysplastic syndromes (MDS) are clonal haematopoietic stem cell disorders characterised by ineffective and dyspoietic haematopoiesis. The natural history of these disorders is variable and ranges from a chronic to a rapid course towards leukaemic progression. Certain shortcomings have been encountered in the French-American-British (FAB) classification over the years, and therefore there is a need for an alternative method of classification. In 1999, the WHO published a revised classification of MDS. In the present study, we have analysed the clinical, haematological and histomorphological features in 96 cases of primary MDS seen in the department of haematology at the All India Institute of Medical Sciences (AIIMS) over a 6-yr period (1996-2001). Both FAB and WHO classifications have been incorporated and the Bournemouth scoring system applied in each case at presentation. The Bournemouth scoring system, in the absence of a cytogenetic study, offers a good prognostication and long-term survival estimate.

Adolescent↗
PubMed1999

Danazol therapy in cyclic acquired amegakaryocytic thrombocytopenic purpura: a case report.

Cyclic acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare disorder characterized by periodic fluctuations in the platelet counts due to a defect in the platelet production. We describe a 42-year-old female with cyclic AATP, in whom the cyclic fluctuations in the platelet counts ceased with danazol therapy. The pathogenesis of the disease and the possible mechanisms of danazol action have been reviewed.

Adult↗
PubMed1999

Visceral leishmaniasis: an opportunistic infection in haematological malignancy.

Visceral leishmaniasis is a rare but potentially life threatening opportunistic protozoan infection in immunocompromised patients. The clinical manifestations in these patients are unusual and the diagnosis is difficult. They need prolonged treatment and are liable to have relapses. Here we report three patients with haematological malignancy (one with acute lymphoblastic leukaemia, one with chronic myeloid leukaemia, and one with myelodysplastic syndrome) complicated with visceral leishmaniasis. The clinical presentation, diagnosis, and outcome are discussed.

Adult↗
PubMed1998

Foetal liver infusion in a chronic myeloid leukemia patient with busulphan toxicity.

A 36-year-old man suffering from chronic myeloid leukemia (in chronic phase) was initially treated with busulphan. At the end of 6 months of follow-up he developed bone marrow aplasia. He was given single foetal liver infusion therapy. The patient recovered fully from aplasia. He continued in chronic phase for more than 7 years with intermittent busulphan therapy.

Adult↗
PubMed1998

Masked polycythaemia vera in a patient with extrahepatic portal venous obstruction.

Myeloproliferative disorder (MPD) is an important cause of thrombosis of the hepatic and portal venous system. The diagnosis in many of these patients is missed as they have an atypical clinical presentation and may have a normal haematological profile at presentation. We report a 30-year-old patient with features of portal hypertension due to extrahepatic portal venous obstruction. She had a normal haemoglobin level and haematocrit at presentation, but the red cell mass was found to be elevated even in the presence of low serum iron levels. A diagnosis of polycythaemia vera as the underlying disease was made.

Adult↗
PubMed1997

Haemopoietic cell composition of human fetal liver, spleen and thymus.

The haemopoietic cell composition of fetal liver, spleen and thymus was studied in human aborted fetuses of 12-22 weeks gestation. Erythroid cells were present primarily in liver and to a lesser extent in spleen. Orthochromatic normoblasts were the predominant erythroid component. Lymphoid cells were seen primarily in the thymus and to a lesser extent in the spleen. There were few granulocytic and megakaryocytic cells in all these organs. A few primitive haemopoietic cells (Haemocytoblasts) were identified only in liver. These observations indicate that during mid-fetal life (12-22 weeks) the liver contributes primarily to erythropoiesis, the thymus to lymphopoiesis, the spleen to both, and that there is a lack of granulopoiesis and megakaryopoiesis at these sites.

Hematopoiesis↗
PubMed1997

Multilineage hemopoietic stem cell defects in Budd Chiari syndrome.

BACKGROUND/AIMS: Erythropoietin-independent endogenous growth of erythroid colony from bone marrow cells has been shown in many patients with Budd Chiari syndrome in earlier studies. In another report, increased megakaryocyte colony growth has also been documented in this disease. However, defects in granulocyte-macrophage cell lines in Budd Chiari syndrome have yet to be reported in the literature. METHODS: Both in vitro erythroid and granulocyte-macrophage colony cultures from peripheral blood mononuclear cells with and without erythropoietin or granulocyte-macrophage colony stimulating factor, respectively, were studied in 32 patients with Budd Chiari syndrome, along with 20 normal healthy controls and ten patient controls with portal hypertension (five patients each with noncirrhotic portal fibrosis and liver cirrhosis). In 18 patients the occlusion was only in the inferior vena cava, in five patients only in hepatic veins, and in nine patients both inferior vena cava and hepatic veins were blocked. RESULT: Endogenous erythroid or granulocyte-macrophage colony growth was not observed in any of the normal healthy controls or in patient controls with portal hypertension. However, 22 of the 32 (68.8%) patients showed endogenous erythroid colony growth. Moreover, four of them also showed endogenous growth of granulocyte-macrophage colony, not previously reported in Budd Chiari syndrome. CONCLUSION: It may be inferred that stem cell defects affecting all three hemopoietic cell lines (erythroid, megakaryocyte and granulocyte-macrophage) occur in Budd Chiari syndrome, which may be the primary defect responsible for the pro-thrombotic state causing venous thrombosis in them.

Budd-Chiari Syndrome↗
PubMed1996

Plasma platelet aggregating factor and platelet aggregation studies in pre-eclampsia.

OBJECTIVE: A plasma platelet aggregation factor (PAF) has been implicated in the pathogenesis of platelet activation in thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Similar mechanism may be operative in pre-eclampsia. METHODS: Coagulation profile and detailed in vitro platelet aggregation with various agonists were studied. PAF was demonstrated by spontaneous aggregation of normal platelets with test plasma. Non-parametric Wilcoxon's rank sum test and Krauskal Wally's one way analysis of variance were applied. RESULT: Twenty-two pre-eclamptic patients and 20 normal pregnant controls were studied. Anti-thrombin III levels were within normal range and fibrin degradation products were only border line raised ( > 10 < 40 micrograms/ml) in 14 (65.4%) patients. In vitro platelet aggregation was abnormal in 17 (77.2%) patients. PAF was demonstrable in 10 of 22 (45.5%) patients. CONCLUSION: Platelet aggregation studies indicated the presence of both activated (hyperaggregable) as well as exhausted (hypoaggregable) platelets in circulation. PAF demonstrable in 45.4% pre-eclampsia patients would suggest its role in the pathogenetic mechanism of platelet activation in this disease.

Adult↗