Ankur Ahuja

Unusual Hairy Projections in a Case of T-acute Lymphoblastic Leukemia, a Cause for Diagnostic Dilemma: A Case Report

T lymphoblastic leukaemia (T-ALL) is a neoplasm of lymphoblasts committed to T-cell lineage and T-ALL is diagnosed by identification of immaturity markers and the lineage specific Cluster of Differentiation (CD) marker, CD3 in the leukemic cells. Here we report a 20 year old male who was diagnosed as case of T-ALL, whose leukemic blasts on morphology resembled the lymphoid cells seen in hairy cell leukemia (HCL) resulting in a diagnostic dilemma which was resolved subsequently by immunophenotyping. Even though the presence of cells with hairy projections is rarely reported in haematological malignancies other than HCL, ours is a first of its kind in TALL.

Unusual massive bone marrow fibrosis in acute promyelocytic leukemia following arsenic trioxide therapy

Bone marrow fibrosis has been associated with different types of non-neoplastic conditions like granulomatous and autoimmune diseases and a variety of neoplastic disorders such as acute megakaryoblastic leukemia, Hodgkin lymphoma, non-Hodgkin lymphoma and myeloproliferative neoplsms. Therapy induced fibrosis is a rare phenomenon. Here we report a case of an incidentally diagnosed acute promyelocytic leukemia (APL) with t(11;17) which was treated with arsenic trioxide (ATO) for 45 days. However, the patient did not go into remission and developed massive fibrosis of bone marrow. Literature search does not reveal such documented marrow fibrosis following therapy with ATO in a case of APL.

Summary and Review of the Abstracts on Philadelphia-Negative Myeloproliferative Neoplasms Presented at Haematocon 2017

There are lot of grey zones in Philadelphia negative chronic myeloproliferative neoplasms (CMPNs) and that’s the reason they are in hit list of researchers. Having a spectrum of disorders their diagnosis is very important and especially to differentiate from each other since they overlap with each other in many ways. Diagnosis doesn’t start from lab but with clinical phenotype. Clinical phenotype not only able to provide us the diagnosis but also helps in management of the disease per se. When diagnosis comes, the old timer but an evergreen morphology plays an important role which along with the newer generation tool “molecular” helps in differentiating these disorders. Lot of studies have already come up from the world. Indian data has also started coming up. When we say about the Indian data nothing holds more important role than Indian Society of Haematology and Blood Transfusion, ISHBT. This small review will cover all papers with BCR-ABL negative CMPNs which were presented at the annual national conference of the ISHBT (Haematocon 2017) which was conducted at Guwahati. These abstract papers from various reputed institutes and centres will provide a short academic journey towards ongoing research activities at these places and will able to guide us regarding Philadelphia negative CMPNs and also stimulate our brain for some left or conflicted areas.

Exteriorisation of the Ommaya Reservoir Secondary to Wound Dehiscence in a Case of ALL

A 41-year-old male a case of acute lymphoblastic leukaemia with C3 disease (CNS involvement) was being managed with modified BFM-90 protocol. For his CNS positive disease, he was planned for intensive triple intrathecal therapy. For the ease of administration of intrathecal and to ensure adequate distribution of chemotherapeutic agents, he was subjected to Ommaya reservoir placement. There were no immediate postoperative complications. The patient received 04 intrathecal therapies through the reservoir as inpatient and was subsequently discharged after CSF was negative and managed as an outpatient. He presented to OPD on D40 of Ommaya insertion with complaints of wetness of the scalp near the site of insertion. Physical examination revealed exteriorisation of Ommaya with CSF leak. CSF examination revealed no evidence of meningitis. Patient hasn’t received any radiation to lead to wound dehiscence. An Ommaya reservoir is a synthetic dome that is surgically placed beneath the scalp and attached to a catheter directed into a ventricle. Complications associated with Ommaya reservoir include technical complications such as misplacement of the catheter, intraventricular haemorrhage, malfunctioning reservoirs, and bacterial meningitis [1]. Other complications included wound dehiscence, ventricular catheter associated cerebral oedema and leakage of cerebrospinal fluid. With wound dehiscence, a superficial wound infection can easily track to the CSF and intracranial cavity resulting in serious intracranial complications. Countersinking is a good technique to prevent wound dehiscence, device extrusion and in irradiated patients with very thin skin, it also enables tension-free closure of the wound [2]. Our patient had wound dehiscence and CSF leak with our associated meningitis in the absence of radiation to scalp at D40 which is an uncommon complication. It is thus mandatory to do a regular local physical examination of the scalp in a patient with Ommaya reservoir (Figs. 1, 2).

Diagnosis of Paroxysmal Nocturnal Hemoglobinuria: Recent Advances

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder with its protean clinical manifestations. This is due to partial or complete absence of ‘glycophosphatidyl-inositol-anchor proteins’ (GPI-AP). The main aim of this review is to highlight various diagnostic modalities available, basic principle of each test and recent advances in the diagnosis of PNH. Recently among various tests available, the flow cytometry has become ‘the gold standard’ for PNH testing. In order to overcome the difficulties encountered by the testing and research laboratories throughout the world, International Clinical Cytometry Society has come up with guidelines regarding the indications for testing, protocol for sample collection, processing, panel of antibodies as well as gating strategies to be used, how to interpret the test and reporting format to be used. It is essential to test at least two GPI-linked markers on at least two different lineages particularly on red cells and granulocytes/monocytes. The fluorescent aerolysin combined with other monoclonal antibodies in multicolour flow cytometry offered an improved assay not only for diagnosis but also for monitoring of PNH clones. It is equally important to diagnose this rare entity with high index of suspicion.

Erratum to: High frequency of infection with tuberculosis as the most common cause of bone marrow necrosis: a study from tertiary care centre in northern India and their clinico-pathologic analysis

The original version of this article unfortunately inadvertently deleted the other authors which is now corrected in the authorgroup of this article.

Prevalence of JAK2V617F mutation in deep venous thrombosis patients and its clinical significance as a thrombophilic risk factor Indian perspective

Venous thromboembolism is known to be a complex interaction of genetic and acquired factors leading to thrombosis. JAK2V617F mutation is believed to contribute to a thrombophilic phenotype, possibly through enhanced leukocyte–platelet interactions in myeloproliferative neoplasms (MPNs). Several studies have focused on the importance of screening for JAK2V617F mutation in patients with splanchnic venous thrombosis (VT) for the detection of nonovert MPNs. The role of JAK2V617F mutation in VT outside the splanchnic region is still widely unsettled. The primary aim of this study was to find out the prevalence of JAK2V617F mutation in patients with deep venous thrombosis (DVT), its clinical significance as a prothrombotic risk factor, and its possible interactions with other genetic thrombophilic risk factors. A total of 148 patients with idiopathic, symptomatic DVT were evaluated. Median age of presentation was 32 years (range 15-71 years) with a sex ratio of 1.3:1. Overall, the most common genetic prothrombotic factor was factor V Leiden mutation, found in 10.8% (16 of 148) of patients who also showed strong association with increased risk of thrombosis (odds ratio 5.94, confidence interval 1.33-26.4, P = .019). Deficiencies in protein C, protein S, and antithrombin were seen in 8 (5.4%), 10 (6.7%), and 8 (5.4%) patients, respectively. It was observed that the frequency of JAK2V617F mutation was lower in Indian patients, and it also showed weaker association with risk of thrombosis, at least in cases of venous thrombosis outside the splanchnic region.