Acute lymphoblastic leukemia as second primary tumor in a patient with retinoblastoma.

Second primary tumor (SPT) is defined as a second tumor that presents either simultaneously or after the diagnosis of an index tumor. Second primary malignancies are the leading cause of death in patients with heritable retinoblastoma (RB). Acute lymphoblastic leukemia (ALL), as SPT in RB patients, is extremely rare. To the best of our knowledge, only five cases of ALL as SPT in patients with RB has been documented in the literature. Herein, we report a case of a 6-year-old girl with bilateral RB, who developed ALL during the course of treatment of RB. This case highlights the importance of reviewing blood investigations regularly to diagnose leukemia as SPT in RB and also the necessity for proper counseling and lifelong follow-up in these patients.

Autoimmune Myelofibrosis in Systemic Lupus Erythematosus Report of Two Cases and Review of the Literature.

Autoimmune myelofibrosis (AIMF) is a rare entity of steroid-responsive bone marrow fibrosis that accompanies a variety of autoimmune diseases, particularly systemic lupus erythematosus (SLE). Rarely it may occur in patients with autoimmune markers but no definable autoimmune disease (Primary-AIMF). We report the cases of two young women with SLE-associated AIMF (SLE-AIMF). The first patient was a young woman who had pancytopenia, massive splenomegaly and reticulin fibrosis in the marrow biopsy. The pancytopenia and splenomegaly resolved completely within weeks of treatment with corticosteroids. Repeat marrow biopsy showed marked regression of marrow fibrosis. The second patient was a young woman with fever, anasarca, bicytopenia and reticulin fibrosis in the marrow biopsy. Steroid therapy resulted in rapid clinical improvement and resolution of pancytopenia. A review of the literature revealed a total of 30 patients with SLE-AIMF reported to-date. Patients with SLE-AIMF are young women with SLE and blood cytopenia who are found to have increased bone marrow reticulin on marrow biopsy. Steroid therapy results in rapid hematological recovery and regression of marrow fibrosis. Whether AIMF is one of several hematological complications of SLE, or represents a unique and distinct subset of patients with SLE in not clear. Prospective studies with longer follow-up are needed to better define the prevalence and clinical spectrum of SLE-AIMF.

Therapy related myelodysplasia/myeloproliferative neoplasia-unclassified with acute leukemic transformation following Paclitaxel and Carboplatin based chemotherapy in an ovarian cancer patient.

Alkylating agents used in chemotherapy are mutagenic and have strong leukemogenic potential. The most serious long term complication of chemotherapy is the development of secondary disease, particularly hematological malignancy; they have rarely been reported in the context of ovarian cancer treatment. We describe quite a rare occurrence of a myelodyplastic/myeloproliferative neoplasm, unclassified (MDS/MPN-U) with acute leukemic transformation and multiple cytogenetic abnormalities not usually found together as JAK2 V617F mutation, 5q- and 7q-deletion, after exposure to paclitaxel and carboplatin based chemotherapy in a patient treated for ovarian cancer. We should be aware of such complication whose prognosis is really poor.

Laboratory diagnosis of Japanese encephalitis using monoclonal antibodies and correlation of findings with the outcome.

Detection of virus, viral antigen, and class-specific antibody was carried out in cerebrospinal fluid (CSF) and sera of 27 children with Japanese encephalitis. The diagnosis could be confirmed in 78.57% of cases (22/27) by demonstration of virus-specific IgM in CSF (15/22), viral antigen in CSF (5/22), or by virus isolation (2/22). Absence of virus specific IgM in CSF was associated with a fatal outcome (P = 0.05).