Evaluation of Rosners index vs Brandt correction and Chang’s %, in the interpretation of mixing studies at varying dilutions

Background: For evaluation of unexplained prolongation of PT and PTT, mixing tests forms a great diagnostic tool. On mixing equal volume of patient plasma with normal pooled plasma, if there is correction it indicates factor deficiency and non-correction indicates inhibitors.Methods: Sysmex CS-5100 Coagulometer with Pathrombin SL APTT reagent, LA1 and LA2 reagents supplied by siemens were used. All data were expressed as Mean±SD. Statistical analysis was done using unpaired students t test. A p value of <0.05 was used to indicate statistical significance in all analyse.Results: APTT with (1:1) and (4:1) mixing study for detection of factor deficiency showed a sensitivity of 91% and 92% for RI, 88% and 90% for Changs %, and 75% for Brandt correction PNP aPTT + 5 secs respectively. For Inhibitors, RI shows a sensitivity of 79% and 89%, Changs 71 and 80% and Brandt test 50% for APTT (1:1) and (4:1) mix, respectively.Conclusions: Mixing tests forms an important diagnostic tool in differentiating factor deficiency from inhibitors especially in LAC patients. This study recommends mandatory use of mixing tests in LAC cases as also advocated by BSH, ISTH and CLSI. Rosners Index is more sensitive than changes % and Brandt correction in the interpretation of mixing studies. It can be safely concluded that RI can be used as a reference method for evaluation of mixing studies and its sensitivity is greatly increased by using PP4:1 PNP. It’s a matter of debate that whether these indices can be effective with other Analysers and reagents?

Comparative evaluation of efficiency of HbA1C, fasting & post prandial blood glucose levels, in the diagnosis of type-2 diabetes mellitus and its prognostic outcome.

Background: HbA1C a marker of chronic hyperglycemia, is associated with diabetes and its complications and has been recommended as a diagnostic test. It is an indicator of average blood glucose concentration over the period of 2-3 months. The main objective of this study was to compare the efficiency of HbA1C, fasting & post prandial blood glucose levels, in the diagnosis of type- 2 diabetes mellitus. Methods: This study was conducted in a tertiary care referral hospital. Total 500 subjects included. Results: The study and control group were almost of the similar ages. FBS & 2 hour PP of control groups are 95.5 ± 9.8 & 168.45 ± 22.8 (mg/dl) respectively & that of type 2DM is 198.5 ± 25.6 & 295.8 ± 32.6 respectively. The HbA1C % of all the 30 cases of DR & all the cases with micro-albuminuria was >7.5%. Conclusions: HbA1C can be used effectively for the diagnosis of type 2 DM & it can be used for predicting the complications of type 2 DM. It shows a direct & linear correlation with the diabetic retinopathy and micro-albuminuria. It is very safe to say that HbA1C is better parameter than FBS & 2 hour PP BS level in diagnosing & predicting the complications of diabetes.

Evaluation of bone marrow aspirate in paediatric patients with pancytopenia: a 2 years study.

Background: Pancytopenia is an important clinico-hematological entity, characterized by a triad of Anemia, leucopenia and thrombocytopenia. It’s not a disease entity itself but a manifestation of many serious and life-threatening diseases. The criteria for defining Pancytopenia is Hemoglobin (HGB) <9 g/dL; total leukocyte count (TLC) <4,000 / μL or absolute neutrophil count (ANC) is < 1.5x 109/ L; platelet count, <100 x 109/ L. The main objective of this study is to classify pancytopenic cases on etiological basis. Methods: A total of 750 bone marrow smears of pancytopenic patients were studied. Results: It comprised of 72 % boys & 28 % girls with the highest number of cases < 5 years of age (64%).The maximum number of cases were of ALL and Aplastic anemias. Conclusions: CBC, clinical findings and PBS provides valuable information in the workup of pancytopenic patients and help in planning additional investigations on bone marrow samples. Bone marrow evaluation is a valuable diagnostic procedure which may confirm the diagnosis of suspected cytopenias. So the take home message is a) pediatric leukemias unlike adults, usually presents with pancytopenia b) CBC+ PBS + BMA with flow cytometry can help in diagnosing majority of pancytopenias.

Evaluation of performance of various histological grading systems of soft tissue sarcomas and the prognosis (metastatic risk and survival rate).

Background: Histologic grade represents the most important prognostic factor for all soft tissue sarcomas and it is strongly associated with the advent of metastasis and patients survival. The main objective of this study is to test individual grading system with metastatic risk and patients survival rate (prognosis). Methods: Soft tissue sarcomas (250) were graded by FNCLCC, NCI & Mhyre Jensens grading system. Special stains & immunohistochemistry were employed whenever necessary. Results: FNCLCC system shows Grade 1 = 50 (20%), Grade 2 = 75 (30%) & Grade 3 = 125 (50%). NCI (Costa et al.) showed Grade 1 = 70 (28%) Grade 2 = 85 (34%) & Grade 3 = 95 (38%). Myhre Jensen showed Grade 1 = 84 (33%), Grade 98 (39%) & Grade 3 = 68 (27%). Undoubtedly, FNCLCC system is the best of all grading systems which is very well supported by statistical analysis in this study. Conclusions: FNCLCC grading system of soft tissue sarcomas is the best documented and tested system. This present study strongly recommends FNCLCC grading system of soft tissue sarcomas to be internationally accepted because the grading system has well defined criteria & so least possible chances of interobserver variability. The present study & few other previous studies highly recommend the mandatory use of FNCLCC grading system in histopathology report format.

Platelet indices- evaluation of their diagnostic role in pediatric thrombocytopenias (one year study).

Background: Thrombocytopenias can be categorised into hypoproliferative (group I) & hyperdestructive types (group II) based on their etiology. Platelet indices (MPV, PDW, P-LCR, platelet-crit) can be used to differentiate this type of thrombocytopenias & these are simple, cost-effective, noninvasive & reliable. The main objective of this study was to evaluate the efficiency of platelet indices to differentiate hypoproductive type from hyperdestructive thrombocytopenias. Methods: Automated Hematology Analyzer Sysmex XT-2000i used to assess platelet indices. 100 Cases of thrombocytopenia & age adjusted (similar age group) controls with normal CBC & peripheral blood smears were included in the study. The gender was not taken into account as the ranges of platelet indices are almost the same for boys & girls of similar age groups. Results: The platelet indices of group I was platelet count = (51.8 ± 31.6) x103/mm, MPV = (8.5 ± 1.27) fl, PDW = (14.10 ± 1.15) fl, P-LCR = (31.90 ± 3.46)%. The platelet indices of group II was platelet count = (39.6 ± 32.7) x103/mm, MPV = (11.6 ± 2.25) fl, PDW = (15.16 ± 1.36) fl, P-LCR = (34.30 ± 2.2)%. Comparative analysis of MPV, PDW & P-LCR of group I and group II showed p value <0.05 proving it to be statistically significant. Conclusions: The combined interpretation of MPV, PDW & P-LCR by automated cell counters can be very useful parameters in differentiating thrombocytopenias due to various etiologies. Platelet indices showed inverse relationship with platelet count as they are increased in hyperdestructive type & shows linear relationship in hypoproliferative type. MPV, PDW & P-LCR can be precisely used to differentiate hyperdestructive type (ITP) from hypoproliferative type (acute leukemias, aplastic anemias). Platelet-crit & platelet large cell ratio are less sensitive parameters to differentiate these thrombocytopenias.

Chediak Higashi Syndrome masquerading as acute leukemia / storage disorder - A rare case report.

Chediak higashi Syndrome (CHS) is a rare autosomal recessive multisystem disorder with a defect in granule morphogenesis with giant lysosomes in leucocyte and other cells. CHS is a rare disease, approximately 200 cases have been reported so far. It was described in detail by Chediak in 1952 and Higashi in 1954. 1½ year old male child presented with multiple hypopigment patches on lower extremities, light colored hair, Hepatosplenomegaly and generalised Lymphadenopathy. PBS shows giant prominent liliac to purple granules in neutrophils, band forms, few lymphocytes and monocytes. Bone marrow is hypercellular showing giant prominent gray blue to purple heterogeneous granules often multiple seen in many myeloid precursors, Neutrophils, few lymphocytes and monocytes. Occasional lymphocytes shows single giant liliac inclusions. Erythropoiesis, myeloid series and Megakaryocytes are mildly increased. Hemophagocytosis noted. CHS is characterised by partial oculocutaneous albinism, frequent fatal bacterial infections, bleeding diathesis and peripheral + Cranial nerve palsies. This disorder further culminates into accelerated phase (Lymphoproliferative Syndrome) progressing into pancytopenia. CHS is due to single gene mutation in LYST (CHS) gene localized to 1q chromosome. The diagnostic hallmark of CHS is presence of giant purple to blue violet inclusions in leucocytes. In this study granules are more prominent in Bone marrow than in PBS correlating well with previous studies. Approximately 85% of the cases, of CHS culminates into Accelerated phase showing Lymphohistiocytic infiltration progressing to pancytopenia and death due to infection. The very rare nature of this disease and its grave prognosis merits its reporting.