Acute lymphoblastic leukemia (ALL) is one of the most common malignancies of childhood. Peripheral blood and bone marrow involvement is common. Extramedullary involvement of central nervous system, liver, spleen, skin and testicles are seen. ALL presenting as nephromegaly is exceedingly rare. This case is reported for its rare presentation as nephromegaly. Bone marrow aspirate showed features of acute lymphoblastic leukemia and immunophenotyping by flow cytometry confirmed the diagnosis.

Acute lymphoblastic leukemia ALL is one of the most common malignancies of childhood. Peripheral blood and bone marrow involvement is common. Extramedullary involvement of central nervous system liver spleen skin and testicles are seen. ALL presenting as nephromegaly is exceedingly rare. This case is reported for its rare presentation as nephromegaly. Bone marrow aspirate showed features of acute lymphoblastic leukemia and immunophenotyping by flow cytometry confirmed the diagnosis.

Dear Sir, Reference to the article “Characteristics of anemia in the Elderly: A hospital based study in South India” recently published in your esteemed journal (2011 volume 27(1), pages 26–32). The authors have systematically approached anemia in the elderly Indian population, an area often neglected. The general symptoms of fatigue in these patients being often attributed to advancing age [1]. A very similar study was conducted at our hospital (St. John’s Medical College Hospital, Bangalore) and the findings published [2]. We would like to draw the comparisons between the two studies (refer Table 1). Table 1 Comparison of findings in two studies on geriatric anemia In our study all anemic patients with age above 60 years (n = 236) were included in the study over a 1 year period (2006–2007). All cases with features of any haematological malignancy were excluded unlike the referenced study. The reference range followed for definition of anemia was as advocated by the WHO (hemoglobin of 75 year age group [2]. Similar to the quoted study, we also found the most common type of anemia to be normocytic, followed by microcytic, macrocytic and dimorphic blood pictures. In addition to peripheral smear evaluation, a bone marrow examination was performed in 24.15% cases. Reactive erythroid hyperplasia was a common finding seen in 45.61% cases. Normoblastic maturation was noted in 59.64% cases, micronormoblastic maturation seen in 22.8% and megaloblastic maturation was noted in 12.28% cases. Features of Myelodysplasia was noted in 3 cases. Iron stores were assessed in all the marrow aspirates. Increased iron stores was noted in 56.14% (indicative of infection), while 0.07% cases had normal iron stores. Etiologically, nutritional anemias were the most common and seen in 77 cases (32.62%). Chronic renal failure was another common cause seen in 46 cases. These findings parallel the quoted study [2]. The authors in the said study have very effectively defined the iron deficient population into those with absolute iron deficiency (Ferritin <20 ng/ml) and mild to moderate iron deficiency (Ferritin 20–100 ng/ml). They have also highlighted the importance of investigating these patients with stool for occult blood and subjecting them to gastroscopy to rule out chronic blood loss and gastro intestinal pathologies which was a frequent finding in their study (22 upper GI and 5 colonic pathologic cases) [1]. In our study the bone marrow iron stores were used as an indicator of iron stores. Depleted iron stores were noted in 29.82% cases. Other parallel studies by Milman and Schultz-Larsen [3] reported lower prevalence of iron deficient patients (2.4% with depleted iron and 3.5% with small/minimal iron stores). As discussed the iron deficient status and anemia per se are seen more commonly in our country as seen in both the studies and other studies done in India. Prevalence of anemia in rural populations in Tamil Nadu is as high as 52% [4]. This could be explained due to lack of awareness in these individuals. In contrast, studies done in the United States (3rd national health and nutrition examination survey (NHANESIII)) to assess the prevalence of anemia in the elderly (aged >65 years), showed the prevalence to be approximately 10% [5], the rate being much lower to that in developing countries like India. In addition the NHANESIII study was done on non institutionalised individuals, unlike our studies (hospital based) [2]. Nutritional deficiencies contributed to 34%, anemia of chronic diseases (ACD) 20% and chronic renal failure (CRF) 8, 4% had both ACD and CRF. In the remaining 34% cases the cause was unexplained [5]. In our study 33 cases (14%) had unexplained anemia [2]. In conclusion, anemia in the elderly is a very prevalent condition in our country. Though nutritional anemias are still common in India, the finding of a normocytic anemia is the most frequently encountered type. As anemia may be only the presenting feature in these patients harbouring more serious pathologies, they have to be approached in a very systematic manner, in order to improve the quality of life and lifespan of the geriatric population.

Sir, Hemolytic disease of newborn (HDN) is defined as neonatal anemia and hyperbilirubinemia caused by an incompatibility between maternal and fetal red blood cells (RBCs).[1] In 98% cases it is caused due to ABO and Rh incompatibility and antibodies to other blood group antigens (Kell, c, E, C, Kidd, Duffy, M, and so on) are causative in remaining 2%.[2] More than 43 different RBC antigens have been reported to be associated with HDN.[3] Red cell antibody screening (RCAS) is a valuable tool in the detection of alloantibodies to other blood group systems (other than ABO and Rh) in the serum of patients during pregnancy or prior to transfusion. Red cell antibody identification (RCAI) should then be carried out on a larger panel of RBCs to precisely identify the antibody. In a prospective study carried out on 624 antenatal cases, RCAS was done using a 3-cell panel from Diamed, Switzerland. RCAI was carried out on cases that were positive for RCAS. RCAS was positive in 9 out of 624 cases—1.4% (excluding the 3 cases who had autoantibodies). After RCAI these were identified as anti-D antibody (6 cases, 66%), anti-D with anti-C antibody (2 cases, 22%), and anti-M antibody (1 case, 11%). The most common antibody identified remained anti-D. In 2 cases of Rh negative pregnancy, the RCAS was suggestive of anti-D. RCAI done, however, showed a combination of anti-D and anti-C. One case of anti-M was detected in a G2P1L1D1 lady. The first pregnancy was full-term normal delivery at home, however, the baby died after birth. The mother’s and baby’s blood group was O positive. RCAS done during second pregnancy was suggestive of anti-Duffy (Fya) or anti-M antibody. RCAI done showed anti-M antibody with dosage effect. The second pregnancy was postdated with Intrauterine growth retardation (IUGR,) and Lower segment caesarean section(LSCS) was done for fetal distress. The baby had hyperbilirubinemia and was Direct Coombs test (DCT) positive requiring phototherapy. Rh incompatibility continues to be a common cause for HDN. Patients with no prior history of sensitization can also develop anti-D as seen in 3 of our cases probably due to naturally occurring anti-Rh antibodies or antepartum hemorrhage. Despite the use anti-D immunization, 1%–2% of the cases are still sensitized. Anti-D immunization resulted in a favorable fetal outcome in the study. Anti-D complicated with anti-C presents with more severe HDN as seen in 1 patient who had a previous stillbirth and a hydrops baby despite receiving anti-D. Anti-C antibodies resulting in HDN requiring exchange transfusion have been reported.[4] Anti-M as a cause of HDN is rare as they are usually complete cold antibodies (IgM). However, it can be IgG type resulting in HDN.[3] HDN due to anti-M antibody can be mild to severe with stillbirth and cases requiring exchange transfusion have been reported.[5] Anti-M causing blood group discrepancy and crossmatch incompatibility has been reported in the Indian literature.[6] Antenatal detection of the non-anti-D causes of HDN requires RCAS. If RCAS is positive, the following steps are to be taken. RCAI should be done to identify the antibody. The spouse has to be screened for the presence of offending antigen and the pediatrician has to be alerted about delivery of a potentially sensitized infant. The blood bank should find a suitable antigen-negative donor for transfusion to baby and mother.

A positive direct Coombs test (DCT) is the hallmark of diagnosis of immune hemolytic anemias. The reagent used for the test is the Antihuman globulin (AHG), which may be either 'Polyspecific' or 'Monospecific'. The advent of the Gel card systems has made the procedure and interpretation of DCT simpler. Aim of this study is to evaluate three of the various techniques used for the performance and interpretation of DCT. A total of 96 EDTA samples were included in the study. DCT was performed by (i) polyspecific AHG manual tube method (ii) polyspecific AHG Gel card method and (iii) monospecific AHG (Anti IgG and Anti Complement) manual tube method. In our study we considered positivity by monospecific AHG as the standard for diagnosis. Of the total 96 samples evaluated, 44 cases positive by Gel card method, were also positive for either one or both the monospecific AHG reagents. 17 cases positive by Gel card were negative by all manual methods. These false positive cases were attributed to reasons such as increased ESR, macrocytosis and marked leucocytosis. Nine cases were negative by Gel card but were positive with the Monospecific AHG. The sensitivity of DCT done by the Gel card technique was 83.01% and the specificity was 60.46%. Use of Gel card technique to perform and interpret DCT is easier than manual tube methods, but positivity by Gel card needs to be correlated with clinical presentation of the patient and other laboratory findings. Monospecific antisera can be used to confirm cases that are positive by the Gel card systems.

OBJECTIVES:The study was undertaken to correlate the blood groups and clinical presentations in malaria patients and to understand the differential host susceptibility in malaria. METHODS:From October 2007 to September 2008, malaria positive patients' samples were evaluated in this study. Hemoglobin, total leukocyte count, and platelet count of each patient were done on an automated cell counter. After determining the blood groups, malarial species and the severity of clinical course were correlated. RESULTS:A total of 100 patients were included in the study, of which 63 cases were positive for Plasmodium falciparum and 37 cases were positive for P. vivax infection and 11 patients had mixed infection. The results of the blood groups showed 22 - 'A' group, 42 - 'B' group, 35 - 'O' group and 1 was 'AB' group. When the clinical courses between different groups were compared using the following parameters for severe infection--a parasitic load of >10/1000 RBCs, severe anemia with hemoglobin < 6 g%, platelet count of <10,000/mm3, hepato or splenomegaly or clinical signs of severe malaria such as fever >101°F and other organ involvement, it was observed that 'O' group had an advantage over other the groups. The difference in rosetting ability between red blood cells of different 'ABO' blood groups with a diminished rosetting potential in blood group 'O' red blood cells was due to the differential host susceptibility. CONCLUSION:'O' group had an advantage over the other three blood groups. Based on literature and the results of this study, the diminished rosetting potential in blood group 'O' red blood cells is suggested as the basis for the differential host susceptibility.

BACKGROUND:Autoimmune hemolytic anemia (AIHA) results from red cell destruction due to circulating autoantibodies against red cell membrane antigens. They are classified etiologically into primary and secondary AIHAs. A positive direct antiglobulin test (DAT) is the hallmark of diagnosis for AIHA.METHODS AND RESULTS:One hundred and seventy-five AIHA cases diagnosed based on positive DAT were included in the study. The cases showed a female predilection (M: F = 1:2.2) and a peak incidence in the third decade. Forty cases were found to be due to primary AIHA, while a majority (n = 135) had AIHA secondary to other causes. The primary AIHA cases had severe anemia at presentation (65%) and more often showed a blood picture indicative of hemolysis (48%). Forty-five percent of primary AIHAs showed positivity for both DAT and indirect antiglobulin test (IAT). Connective tissue disorders were the most common associated etiology in secondary AIH A0 (n = 63).CONCLUSION:AIHAs have a female predilection and commonly present with symptoms of anemia. AIHA secondary to other diseases (especially connective tissue disorders) is more common. Primary AIHAs presented with severe anemia and laboratory evidence of marked hemolysis.

Dear Editor, Glucose-6-phosphate dehydrogenase deficiency is the most common enzyme deficiency worldwide. Approximately 400 million people are said to be affected worldwide. According to world health organization 7.5% of world population are carriers of glucose-6-phosphate dehydrogenase deficiency and 2.9% were G6PD deficient [1]. It causes spectrum of diseases including neonatal hyperbilirubinemia, acute hemolytic and chronic hemolytic anemia. Usually the affected individuals are asymptomatic unless they are provoked by oxidative stress [2]. This enzyme deficiency is very prevalent in individuals of Africa, America, Mediterranean, and East Asia. In India the incidence of G6PD has been variably reported as 0–37% in different castes and communities. Higher incidence of G6PD deficiency is seen in north and west India (15%) as compared to south India (1–2%) [3]. It implies that this inherited metabolic disorder is an important health problem in India and it is necessary to carryout large scale screening in the whole population. Severe manifestations of G6PD deficiency can be prevented if necessary precautions are taken, hence this study was done to determine the incidence of G6PD deficiency in asymptomatic population represented by healthy blood donors and to characterize the demographic profile and laboratory profile of such group. The study was conducted on 2005 healthy blood donor samples. This screening program included 2000 male and 5 female blood donors who came to blood bank for donation of blood. These donors were from different part of India. The G6PD activity was assessed in their red cells using anticoagulated (EDTA) venous blood. Sixteen blood donors out of 2005 screened by methaemoglobin reduction test were found to be G6PD deficient. All of them were male. These individuals were clinically normal and had no significant past history. The incidence of G6PD deficiency was 0.8% in the sampled population. Among this 16 G6PD deficient subjects two blood donors were from West Bengal, one from Kerala and the rest of the 13 subjects were from Karnataka. The mean Haemoglobin values of the normal donor population vs. G6PD deficient donor population were compared using unpaired t test. The difference in the mean Hb value was not statistically significant (P value > 0.5). Sixteen units of blood were transfused to patients. There was two [12.5%] transfusion related reaction among the patients who received the G6PD deficient blood. One of the patients, who was transfused following upper Gastro intestinal bleeding, had itching and rashes all over the body after transfusion of 50 ml blood. The reaction workup done ruled out haemolytic reaction. A probability of allergic reaction was considered. As in this patient transfusion was stopped and he did not receive further transfusions for next 48 h, his Hb fell from 9.0 to 7.7 gm/dl. The second patient was a neonate undergoing surgery for a ruptured meningomyelocoele. The blood was transfused during surgery following which the baby developed mottling and passed red colored urine. A hemolytic transfusion was suspected. The transfusion reaction workup showed haemolysis in the post transfusion sample and hemoglobinuria. But blood bag showed no hemolysis. However as pretransfusion sample also showed haemolysis, a hemolytic reaction could not be conclusively reported. Hemoglobin dropped from 12.2 to 8.0 gm/dl. The beneficial effects of blood transfusion, for the typical 70 kg human each unit of whole blood should increase the hematocrit by 3–5% or hemoglobin by 1–1.5 gm/dl [4].Testing for G6PD deficiency is not routinely practiced for donors units [2]. Various studies have reported a mild increase in bilirubin, LDH, and decrease in hemoglobin following the transfusion of G6PD deficient blood. This is especially in neonates (preterm). Transfusion of G6PD deficient red cells to the premature infants has been associated with hemolytic and severe hyperbilirubinemia requiring exchange transfusion [5]. In our study also one neonate developed suspected hemolytic transfusion reaction following transfusion of G6PD deficient blood. Under normal circumstances the transfusion of G6PD deficient blood is harmless for most of the individuals until they are exposed to certain oxidative drugs. This study was an attempt to find out the prevalence of G6PD deficiency in our population. This will help us to quantitate the magnitude of the problem in the population and initiate measures to screen the population or a selected group in the population. The prevalence of G6PD being very low in this study (0.8%) and as many people remain asymptomatic unless challenged with oxidative drugs or infection, screening the whole population for G6PD deficiency is not warranted. As one of the neonates receiving G6PD deficient blood developed suspected hemolytic reaction, screening of blood to be transfused for select population like neonates is advisable.

Microangiopathic hemolytic anemia (MAHA), is one of the causes of extra vascular hemolysis. It is seen in settings with pathologically altered small blood vessels. Disseminated carcinomas may rarely present as MAHA. A case of a 28 year old female with carcinoma stomach, who presented with MAHA as a first manifestation is reported. Acute onset of MAHA, may be the first manifestation of malignancy. In the absence of relatively common causes like disseminated intravascular coagulation,/Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura, MAHA warrants extensive rapid investigations including bone marrow aspiration for possible metastatic deposits.

Objective: To assess the efficacy of a peripheral smear examination as a screening tool for β-thalassemia trait. Materials and Methods: 17 623 Leishman-stained peripheral smears were evaluated during the period from July 2006 to September 2007. The following parameters were studied: hemoglobin, red blood cell count, mean corpuscular volume, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration and red cell distribution width. All the cases that showed microcytosis, hypochromia, erythrocytosis and absence of anisopoikilocytosis were suspected of having the thalassemia trait (TT), and all these cases were further evaluated with Alkaline Hemoglobin Electrophoresis for confirmation. Results: Of the 17 623 smears examined, 60 cases were considered suspicious of having TT. Alkaline hemoglobin electrophoresis carried out on all these cases revealed an elevated HbA 2 (Mean = 7.5%). Five cases evaluated were found to have other hemoglobinopathies (1 Sickle cell trait, 3 Hb-E, 1 thalassemia intermedia). Conclusion: Careful screening of peripheral smear is an invaluable screening tool for thalassemia trait (PPV - 95%). There must be awareness among the peripheral centers about the importance of peripheral smear screening and the affected persons should be counseled.

A 62-year-old man presented with rapidly growing tumour in the right parotid region with associated pain and facial nerve palsy. Based on the fine needle aspiration cytology report of high-grade mucoepidermoid carcinoma, parotidectomy was performed which showed features of salivary duct carcinoma. The smears were reviewed to identify the potential pitfalls in the cytological diagnosis of salivary duct carcinoma.