Childhood T-lineage Acute Lymphoblastic Leukemia: Management and Outcome at a Tertiary Care Center in North India.

Objective: To assess the clinical features, prognostic factors and outcome of childhood T-ALL in comparison with B-lineage ALL, treated with a uniform treatment regimen (MCP 841). Setting: Pediatric oncology division of a tertiary care institution in Northern India. Design: Retrospective analysis of clinical data and survival outcome. Participants: 60 children with T-ALL and 139 with Blineage ALL, and less than 15 years of age treated over 15 years. Results: T-ALL was observed in 30%. High risk features at presentation (age 10 years, WBC >50,000/mm3, mediastinal mass, and CNS leukemia) were significantly more frequent in T-ALL as compared to B-lineage ALL (P=0.049, P<0.001, P<0.001 and P=0.02, respectively). Fifty five of 60 T-ALL patients (91.7%) achieved complete remission after induction therapy. There were 3 induction and 10 remission deaths while 11 (18.3%) relapsed. The overall survival and event-free survival of T-lineage ALL (61.5±7.6 and 49.9±7.4, respectively) were similar to that of B-lineage patients (68.7±4.7 and 47.1±5.1, respectively). National Cancer Institute risk groups emerged as significant prognostic factor for event free survival only in B-lineage patients. Conclusions: Even though high risk features were significantly more frequent in T-ALL, survival outcome was similar to that of B-lineage patients. None of the routinely described prognostic parameters significantly impacted survival.

Etiology and outcome of oral mucosal lesions in children on chemotherapy for acute lymphoblastic leukemia.

Microbiological cultures were taken from oral cavity and blood in 100 mucositis episodes in 70 children with acute lymphoblastic leukemia (ALL). Oral mucositis was commonest in neutropenic children during induction chemotherapy. Fungal organisms (n=39) were commonest isolate from mucosa followed by bacteria (n=28). Isolation of organism from oral cavity had no association with those isolated from blood. Herpes serology was positive in 16% episodes compared to 2% of controls. Obtaining cultures from oral lesions is useful in appropriate management of lesions and thereby possibly preventing systemic spread.

Invasive colonic mucormycosis in early induction therapy of childhood acute lymphoblastic leukemia.

Fungal infections occur in patients who are severely immunocompromised with profound and prolonged neutropenia. We report a patient of acute lymphoblastic leukemia who developed nonspecific abdominal symptoms within two days after the onset of neutropenia in the early induction phase of chemotherapy, which was later found to be due to intestinal mucormycosis and resulted in a fatal outcome.

Autoimmune hemolytic anemia complicating disseminated childhood tuberculosis.

An 8-year-old girl presented with severe autoimmune hemolytic anemia (AIHA) in association with mediastinal widening. Biopsy of mediastinal lymph node confirmed the diagnosis of tuberculosis. A diagnosis of disseminated tuberculosis in association with AIHA was made, and the patient was treated with steroids and antitubercular therapy. This is the first report case of AIHA in association with childhood tuberculosis; we also discuss other reported cases of AIHA in association with adult tuberculosis in English literature.

Bone relapse in acute lymphoblastic leukemia.

Acute lymphoblastic leukemia (ALL) can occasionally relapse in unusual extramedullary sites like bone. Here we present a 6.5-year old boy with 'T' cell ALL who developed a swelling in left tibia which was infiltrated with lymphoblasts 7 months after completion of chemotherapy. Bone marrow and cerebrospinal fluid were negative for blasts. This is the first reported case of bone relapse in ALL from India. We discuss the previous cases of isolated bone relapse in ALL reported in English literature.

Breast feeding and childhood hematological malignancy.

Breast milk is known to have anti-infective and immunomodulating effects on infants, but its association with childhood cancer has not been well studied. Artificial feeding may affect the immune response in carcinogenesis. In this communication the authors have reviewed different articles describing the association between breast feeding (BF) and subsequent development of childhood hematological malignancy. It appears that BF may have a protective effect on childhood cancer, both the duration of BF as well as the quantity of milk ingested is probably critical to the beneficial immunological effects of BF against childhood cancer if any.

Non hodgkin's lymphoma seven years following remission of acute lymphoblastic leukemia.

The authors describe a case of extramedullary relapse in lymph node presenting as lymphoblastic lymphoma seven years following remission of acute lymphoblastic leukemia. To the best of our knowledge, this is the first reported case of an isolated lymph node relapse with hematopoietic remission of leukemia. We have discussed cases of large cell lymphoma and other unusual areas of extramedullary relapse complicating acute lymphoblastic leukemia in hematopoietic remission.

Transthoracic lung aspiration in etiology of pneumonia.

Transthoracic lung aspiration was performed in 30 episodes of pneumonia in 27 children with malignancy on chemotherapy to assess etiology of pulmonary infections. Total of 22 organisms were isolated in 16/30 (53.3%) episodes. No acid fast bacilli or Pneumocystis carinii were seen. Organisms grown by blood culture correlated with that of lung puncture in 5 episodes, while throat culture and nasopharyngeal organisms correlated with that of lung puncture on one occasion each. Organisms isolated in 8/18 episodes (44.4%) of antemorten transthoracic aspiration included: Pseudomonas aeruginosa, Klebsiella pneumoniae, Escherichia coli, Streptococcus faecalis and Diphtheroids. In 3/18 episodes, lung puncture results altered treatment and thus resulted in survival of the patients. Only one minor complication occurred in this study--pneumothorax that resolved spontaneously. Thus, transthoracic lung puncture is an useful and safe procedure in immunocompromised patients with pneumonia who do not respond to initial broad spectrum antibiotics.

Thrombocytopenic purpura as a presenting manifestation of tubercular lymphadenitis.

A 11-year-old girl presented with thrombocytopenic purpura along with cervical lymphadenopathy. There was no hepatosplenomegaly; no evidence of microangiopathy; bone marrow examination was normal. Acid fast bacilli were seen in fine needle aspiration cytology of the lymph node. A diagnosis of tubercular lymphadenitis in association with immune thrombocytopenia was made, and the platelet count recovered with antitubercular therapy. This report illustrates immune thrombocytopenia as a rare manifestation of childhood tuberculosis; the authors also discuss other causes of thrombocytopenia in childhood tuberculosis.

Acute myeloid leukemia presenting simultaneously in two siblings.

The rare occurrence of acute myeloid leukemia simultaneously in two siblings, 3-year male and 1-year female, in the absence of any known predisposing condition is reported. We speculate that an initial in-utero genetic alteration followed by subsequent environmental exposure to some unknown toxin may have resulted in leukemogenesis.

Adenoid cystic carcinoma of the lacrimal gland.

Adenoid cystic carcinoma (ACC) of the lacrimal gland is a rare tumor from the epithelial structure. It is rare in children. The authors report a case of ACC in a girl child. Extensive excision of the tumor is advised to prevent later recurrences. Local control can be better achieved by a combination of radiochemotherapy as ACC has been shown to recur event after 10 years.

Etiopathophysiology of disseminated intravascular coagulation.

Disseminated intravascular coagulation (DIC) involves activation of clotting as well as fibrinolytic pathways. Thrombosis from thrombin release results in end-organ damage, whereas consumption of coagulation factors results in bleeding. Sepsis is the commonest cause of DIC. The consumption of antithrombin in sepsis abrogates its anti-inflammatory role and so its low level is a poor prognostic marker in sepsis. The increased release of plasminogen activator inhibitor-1 (PAI-1) as seen in sepsis decreases fibrinolysis and promotes increased microvascular thrombosis. Here, we discuss the role of inhibitors of coagulation, cytokines, kinins, complement and vasoactive peptides in DIC.

Diagnosis and treatment of disseminated intravascular coagulation.

Disseminated intravascular coagulation (DIC) is a complex clinical syndrome with activation of the procoagulant and fibrinolytic systems along with inhibitor consumption. We discuss regarding the controversies in diagnosis and management of DIC. Bleeding is a more common manifestation of DIC but most of the morbidity and mortality of DIC is due to microvascular thrombosis. Routinely performed tests for DIC such as platelet count and prothrombin time may be normal in chronic DIC. There is no single test that would diagnose DIC, however, estimation of D-dimer appears to be the most sensitive and specific test. Therapy of DIC aims at treating the primary cause. Fresh frozen plasma and platelet concentrates are recommended only in bleeding patients and have the potential risk of adding procoagulant material to the already activated procoagulant system. Role of heparin and antithrombin in patients with sepsis and DIC is discussed.

Venous thrombosis associated with Salmonella: report of a case and review of literature.

We describe here the first case of Salmonella paratyphi A bacteremia associated with deep vein thrombosis in a 10-year-old patient. In spite of aggressive antibiotic therapy and supportive care, the patient died of progressive respiratory distress and septic shock. Awareness of unusual clinical presentation of Salmonella infection in important. A review of the previously reported cases of Salmonella associated venous thrombosis worldwide is also presented.