A case of paraneoplastic hyperleukocytosis closely mimicking chronic neutrophilic leukemia

Leukemoid reaction and myeloproliferative syndrome are close mimickers and frequently pose a diagnostic dilemma, particularly when the leukocyte count is very high. Leukocyte alkaline phosphatase score frequently aids in diagnosis but may or may not be contributory, especially in differentiating chronic neutrophilic leukemia. Herein, we document a case of leukemoid reaction with extensive hyperleukocytosis in a 46-year-old female with poorly differentiated carcinoma. The tumor itself as well as the associated leukocytosis portends a poor prognosis

De novo Philadelphia chromosome positive myelodysplastic syndrome: Report of two cases with brief literature review

Myelodysplastic syndromes (MDSs) are characteristically defined by the presence of specific karyotypic abnormalities, based on which they have been prognosticated. Translocation t(9;22)(q34;q11.2) (Philadelphia positive [Ph +ve]) and corresponding BCR-ABL fusion transcript is the defining parameter of chronic myeloid leukemia. It is also seen in a fair proportion of adult acute lymphoblastic leukemia. Occurrence of a Ph +ve MDS is very uncommon, and that too is seen mostly on progression to higher stage/acute leukemia. Even rarer is the de novo presence of Ph positivity in an MDS. A literature search through PubMed has shown only about forty cases of Ph +ve MDS among which less than half had shown Ph positivity at the time of initial diagnosis. Due to its rarity, this entity has not yet found its space in current WHO 2008 classification and is still under “yet to be validated phase” in current practice of hematological malignancies. The benefit of using a tyrosine kinase inhibitor in such a situation is also debatable. We report here two such cases of de novo Ph +ve MDS, diagnosed in last 1½ year at our institute along with brief literature review

Microfilariae in bone marrow aspirate of a case of myelofibrosis: A cause or coincidence?

Filariasis is among the common parasitic infestations found in India, with Wuchereria bancrofti being the most common causative organism. Presentation ranges from clinically asymptomatic to profound elephantiasis. It is also detected incidentally in diagnostic samples such as body fluids, fine needle aspirates, peripheral blood smears, and other cytological smears. Its detection in bone marrow aspirates with an associated hematolymphoid neoplasm is rare, with only a few case reports. We report one such case of young male who presented with leukocytosis of 253 × 109/L with basophilia and massive splenomegaly. Bone marrow aspirate smears showed the presence of microfilariae along with other features of a myeloproliferative neoplasm (MPN). The present case is probably the first case of finding a microfilaria in a case of MPN

Efficacy of stem cell in improvement of left ventricular function in acute myocardial infarction - MI3 Trial.

Background & objectives: Acute myocardial infarction (AMI) is characterized by irreparable and irreversible loss of cardiac myocytes. Despite major advances in the management of AMI, a large number of patients are left with reduced left ventricular ejection fraction (LVEF), which is a major determinant of short and long term morbidity and mortality. A review of 33 randomized control trials has shown varying improvement in left ventricular (LV) function in patients receiving stem cells compared to standard medical therapy. Most trials had small sample size and were underpowered. This phase III prospective, open labelled, randomized multicenteric trial was undertaken to evaluate the efficacy in improving the LVEF over a period of six months, after injecting a predefined dose of 5-10 × 108 autologous mononuclear cells (MNC) by intra-coronary route, in patients, one to three weeks post ST elevation AMI, in addition to the standard medical therapy. Methods: In this phase III prospective, multicentric trial 250 patients with AMI were included and randomized into stem cell therapy (SCT) and non SCT groups. All patients were followed up for six months. Patients with AMI having left ventricular ejection fraction (LVEF) of 20-50 per cent were included and were randomized to receive intracoronary stem cell infusion after successfully completing percutaneous coronary intervention (PCI). Results: On intention-to-treat analysis the infusion of MNCs had no positive impact on LVEF improvement of ≥ 5 per cent. The improvement in LVEF after six months was 5.17 ± 8.90 per cent in non SCT group and 4.82 ± 10.32 per cent in SCT group. The adverse effects were comparable in both the groups. On post hoc analysis it was noted that the cell dose had a positive impact when infused in the dose of ≥ 5 X 108 (n=71). This benefit was noted upto three weeks post AMI. There were 38 trial deviates in the SCT group which was a limitation of the study. Interpretation & conclusions: Infusion of stem cells was found to have no benefit in ST elevation AMI. However, the procedure was safe. A possible benefit was seen when the predefined cell dose was administered which was noted upto three weeks post AMI, but this was not significant and needs confirmation by larger trials.

Immunophenotypic patterns in precursor T-cell neoplasm.

T-cell lymphoproliferative disorders are a heterogeneous group of lymphoid neoplasm that can mimic both benign conditions and non-hematopoietic tumors. In routine clinical practice, morphology and immunophenotyping forms the basis of their diagnosis. In this retrospective analysis, we evaluate the utility of flow cytometric immunophenotyping patterns in diagnosis of precursor T-cell neoplasm. Aberrant expression of T-cell antigens was found in all the cases of precursor T-cell neoplasm. The residual normal T-lymphocytes, identifiable in majority of cases, were found to be useful in evaluation of quantitative differences in antigen expression by leukemic cells. A careful analysis of flow cytometric immunophenotyping data can provide additional information which is useful for diagnosis of precursor T-cell neoplasm. This information can be further utilized for analysis of minimal residual disease in these tumors.

Parotid Gland Enlargement as a Presenting Manifestation of Acute Lymphoblastic Leukemia

A young female presented with symmetric polyarthritis, generalized lymphadenopathy, hepatosplenomegaly and bilateral parotid gland enlargement without sicca symptoms. A second case of 15 months old child presented with short duration pyrexia with generalized lymphadenopathy, hepatosplenomegaly and bilateral parotid gland enlargement. Both the patients had out of proportion anemia on examination. Investigations confirmed CD 10+ B-cell acute lymphoblastic leukemia (ALL) in both the cases. Fine needle aspiration cytology of parotid glands in both the cases showed infiltration by lymphoblasts.We propose that ALL should be included in the differential diagnosis of bilateral parotid gland enlargement especially if associated with lymphadenopathy and hepato-splenomegaly.

Nocardiosis in patients of chronic idiopathic thrombocytopenic purpura on steroids.

We present two cases of chronic idiopathic thrombocytopenic purpura (ITP) on prolonged steroid therapy who developed subcutaneous and brain abscesses due to Nocardia asteroides. The special diagnostic and therapeutic challenges encountered in the patients because of severe thrombocytopenia are being highlighted.

Acute Promyelocytic Leukemia : Experience at a North Indian Tertiary Care Hospital with Review of Literature

Acute promyelocytic leukemia (APL) is characterised by balanced translocation between the long arms of chromoso)ne 15 and 17 resulting in formation of fusion protein PML/RARa. Due to this abnormal fusion protein, myeloid cell differentiation is arrested at the promyelocyte level. This molecular defect and myeloid cell differentiation arrest can be overcome by pharmacologic doses of ali-trans retinoic acid (ATRA). APL most common Iy presents as catastrophic bleeding manifestations which is a major cause of mortality. If diagnosed and treated early, patients can be salvaged and can achieve long term disease free survival. Our experience of seven patients is presented. All patiens presented with bleeding manifestation and two died due to it. Rest of the five patients who underwent chemotherapy in the form of induction with ATRA along with supportive measures (fresh frozen plasma and platelets) followed by consolidation therapy in the form of multi-agent chemotherapy, achieved prolonged disease free remission. Thus with early diagnosis and start of ATRA, APL is a potentially curable malignancy.