Outcomes of pediatric mixed phenotype acute leukemia treated with lymphoid directed therapy: Analysis of an institutional series from India.

There is limited data regarding pediatric mixed phenotype acute leukemia (MPAL) and there is no global consensus on its management yet. In this retrospective study, we analyzed the outcomes of children diagnosed with MPAL at our institute. This study included children ≤ 14 years with MPAL who presented to a tertiary cancer center in India from January 1st 2009 to December 31st 2015. Over a seven-year period, 1390 patients with leukemia presented to our institute of which 22 patients (1.5%) had MPAL. Sixteen patients (72.7%) had B/myeloid leukemia, while 4 (18.1%) and 2 (9%) patients had T/myeloid and B/T leukemia respectively. Twenty-one patients were treated with a modified BFM ALL 95 protocol. 76.1% (n = 16) of patients had a good prednisolone response (GPR) on day 8 and end-of-induction (EOI) marrow was in remission in 90.5% (n = 19). A poor prednisolone response (PPR) on day 8 correlated with an inferior relapse-free survival (25% vs 79.5%, P=.025). The 4-year event-free survival (EFS) and overall survival (OS) for the entire group was 60.8% and 64.9% respectively while the EFS for patients who had a GPR and remission at the EOI (n = 15) was 80% as compared to 16.7% in patients with PPR or induction failure. Lymphoid directed chemotherapy is seen to have good survival outcomes in pediatric MPAL. However, a PPR on day 8 or a positive EOI marrow may be an indication for more aggressive treatment.

Primary Mediastinal Large B-Cell Lymphoma in a Child Presenting With Superior Mediastinal Syndrome and Chylous Pleural and Pericardial Effusion.

Primary mediastinal (thymic) large B-cell lymphoma is an aggressive B-cell lymphoma. It comprises <3% of all pediatric non-Hodgkin lymphomas (NHLs). Primary mediastinal (thymic) large B-cell lymphoma usually presents with serous pleural effusion, but presentation with chylous pleural and pericardial effusions is rare. We present a child who presented with features of a superior mediastinal syndrome. Biopsy of the mediastinal mass confirmed the diagnosis of large B-cell lymphoma. In view of nonimprovement of respiratory distress with chemotherapy and persistence of features of superior mediastinal syndrome, the child was evaluated and found to have massive pleural and pericardial effusion on imaging. Therapeutic thoracentesis and pericardiocentesis revealed chylous nature of the fluid.

Profiling of peripheral T-cell lymphomas in Kerala, South India: A 5-year study.

BACKGROUND: Peripheral T-cell lymphomas (PTCLs) are non-Hodgkin's lymphomas (NHLs) with considerable variation in incidence across the world. They show a wide variety of clinicopathological features and generally associated with poor clinical outcome. Lymphoma data from different geographic regions will definitely aid in routine clinical practice and research work. PTCLs are reported with a higher frequency in Asia as compared to Western countries. OBJECTIVE: The objective of this study was to analyze the frequency and distribution of PTCLs diagnosed in a tertiary care cancer center in Kerala. MATERIALS AND METHODS: This was a retrospective study carried out in the Division of Pathology, Regional Cancer Centre, Thiruvananthapuram, for 5 years from January 1, 2011, to December 31, 2015. All PTCLs diagnosed during this period were reviewed and then classified according to the 2016 revision of the World Health Organization classification of lymphoid neoplasms. Statistical significance of the results was evaluated using Chi-square test. RESULTS: Among the total 3108 cases of lymphomas diagnosed at our center, 2404 cases were NHLs (77.35%). PTCLs (n = 333) contributed 13.85% of all NHLs. Among these, PTCL, not otherwise specified, constituted the most common subtype (92 cases, 27.63%), followed by angioimmunoblastic T-cell lymphoma (79 cases, 23.72%), anaplastic large cell lymphoma (75 cases, 22.52%), mycosis fungoides (28 cases, 8.40%), and adult T-cell leukemia/lymphoma (ATLL) (28 cases, 8.40%). CONCLUSION: This is the largest study on PTCLs reported from Kerala. We document that the frequency of PTCLs is higher than that reported from Western studies. The frequency of ATLL reported from Kerala is much higher than that reported from other states.

Mediastinal Mass with Hyper-eosinophilia in a Young Boy -A Diagnostic Dilemma.

Mediastinal masses in children comprises of a heterogeneous group of tumours. In such cases, biopsy and histological analysis are mandatory for planning of treatment. We have reported an unusual aetiology for a mediastinal mass in a young boy presenting with features of Superior Vena Caval Obstruction (SVCO) who also had marked blood and marrow eosinophilia mimicking Chronic Eosinophilic Leukaemia (CEL). We have also discussed the differential diagnoses of mediastinal tumours with hyper-eosinophilia and possible therapeutic implications.

Flow cytometric analysis of Mixed phenotype acute leukemia: experience from a tertiary oncology center.

INTRODUCTION: Mixed phenotype acute leukemia (MPAL) is a rare subset of acute leukemia where the blasts exhibit lineage specific antigens of more than one lineage. Flow cytometric immunophenotyping is essential for the diagnosis of MPAL and the accurate diagnosis highly depends on the panel of markers used. The precise incidence of MPAL is uncertain as various institutions use different combinations of antibodies to assign the blasts to a particular lineage. AIM: The aim was to study the immunoprofile of acute leukemia including aberrant antigen expressions and to study the incidence, clinical features, laboratory findings, and immunophenotype of MPAL in our institution. MATERIALS AND METHODS: All cases of acute leukemias in which flow cytometric analysis during 1-year period from July 2012 to July 2013 were included in this study. RESULTS: During the study period, flow cytometric analysis of 506 cases was performed. B lymphoblastic leukemia was the most common subtype of acute leukemia. CD13 was the most common aberrant antigen expression in acute lymphoblastic leukemia and CD7 was the most common aberrant antigen expression in acute myeloid leukemia. A diagnosis of MPAL was made in 15 cases, which accounted for 2.96% of all leukemias. 9 cases were diagnosed as T/myeloid, 5 cases as B/myeloid and 1 case as B/T. CONCLUSION: Mixed phenotype acute leukemia is a rare subset of acute leukemia. Flow cytometry is critical in establishing a diagnosis of MPAL. The panel of antibodies used is important in the identification of the "mixed" phenotype. Cytoplasmic markers (cytoplasmic MPO, cytoplasmic 79a, cytoplasmic 22 and cytoplasmic CD3) should be included in the primary flow cytometric panel.

Clinical and hematological profile of acute megakaryoblastic leukemia: a 2 year study.

Acute megakaryoblastic leukemia is a rare subtype of acute myeloid leukemia with a characteristic morphologic and immunophenotypic profile. It has to be distinguished from other subtypes of acute myeloid leukemia as well as acute myeloid leukemia with t (1; 22) (p13;q13) and acute megakaryoblastic leukemia in Down Syndrome because of its poor prognosis. We studied ten cases diagnosed over a period of 2 years (from July 2011 to June 2013). All the ten cases were in the pediatric age group ranging from 4 months to 2 years. On morphology, pointers to the diagnosis were clustering of blasts, presence of cytoplasmic blebs and platelet budding. An additional interesting morphological feature observed in our study was nuclear blebs which were seen in nine cases. Diagnosis was confirmed in all cases by positive immunostaining for CD61. Two of the cases had an extremely rare clinical presentation as granulocytic sarcoma. Although rare, acute megakaryoblastic leukemia should be kept in mind especially in leukemia in infants.

Burkitt's lymphoma of the humerus.

Burkitt's lymphoma is an uncommon form of non-Hodgkin's lymphoma (NHL) in adults and represents < 5% of NHL adults. Burkitt's lymphoma involving primarily the appendicular skeleton is rarely described. We present the case of a young man with primary Burkitt's lymphoma involving the humerus as the only site of disease. He received R hyper CVAD and local irradiation and is in complete remission at 24 months.

Primary renal lymphoma-a case report.

Primary renal lymphoma is a rare entity representing less than 1% of lesions in the kidney. We present the case of a 42-year-old male who was evaluated for pain and a mass in the abdomen. The computed tomogram of the abdomen showed a large lobulated homogeneously enhancing mass lesion of about 14×12×18 cm, involving the whole of the left kidney and encasing the left renal vessels and ureter. The patient underwent a biopsy, and the histopathology was diffuse large B cell lymphoma, positive for LCA, CD20, PAX 5, Bcl 2 and negative for SIgM, CD33, CD34, CD5, Tdt, with MIB 1 labelling index of 40%. He received chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (R CHOP) for eight cycles followed by radiation to the residual mass and achieved complete remission. Currently, he is alive and in remission at 28 months.

A colonization of a different kind.

An unusual morphological pattern in the lymph node can at times, pose a diagnostic problem. We report a case of a 55 year old male whose cervical lymph node biopsy showed an unusual pattern of follicular colonization by T-lymphoblasts. The interfollicular area showed a diffuse infiltrate of Langerhans cells. A diagnosis of a T lymphoblastic lymphoma coexisting with Langerhans cell histiocytosis like lesion was made, keeping in mind lack of clinical evidence for the latter.

Solitary plasmacytoma of the metacarpal bone in an adolescent.

Solitary plasmacytoma of the bone (SPB) is a plasma cell neoplasm that usually presents as a lytic lesion mainly localized within the axial skeleton. The occurrence of SPB in young individuals is exceedingly rare, but has been sporadically reported before. We report a case of SPB involving metacarpal bone in a 16-year-old male with a prior history of trauma at the same site.

Extramedullary plasmacytoma of the trachea.

Extramedullary plasmacytomas are plasma cell tumors that occur outside the bone marrow. They constitute around 4 % of all plasma cell neoplasms. The most common site of extramedullary plasmacytoma is the upper aerodigestive tract-nasal cavity, paranasal sinuses and oronasopharynx. We are presenting a case of extramedullary plasmacytoma of the trachea. Trachea is an extremely rare site of plasmacytoma. When extraosseous plasmacytoma occur in uncommon sites, the distinction from B cell lymphomas showing extensive plasmacytic differentiation can be difficult and diagnostically challenging.