Bartonella henselae Infection and Lymphadenopathy in a Patient With T Cell Acute Lymphoblastic Leukemia.

Patients undergoing therapy for T cell acute lymphoblastic leukemia are at risk of infections during their treatment course. Cat scratch disease caused by Bartonella hensalae can masquerade as leukemic relapse and cause systemic infection. Obtaining a thorough exposure history may aid clinicians in making the diagnosis.

Sinusoidal Obstruction Syndrome of the Liver Associated With 6-Mercaptopurine During Maintenance in a Child With T-cell Acute Lymphoblastic Leukemia.

Sinusoidal obstruction syndrome (SOS) of the liver is a complication of chemotherapy most often encountered with hematopoietic stem cell transplant due to high-dose conditioning regimens, but it can also occur with regimens outside of the transplant setting. Mild-to-moderate SOS is a well-described 6-thioguanine toxicity; however, it has rarely been reported as secondary to 6-mercaptopurine, a related thiopurine. This report details a case of a 10-year-old male with T-cell acute lymphoblastic leukemia who developed severe SOS during maintenance therapy with 6-mercaptopurine, and a review of the related literature.

Myeloid and lymphoid neoplasm with novel complex translocation: unusual case report with T-lymphoblastic lymphoma, myeloid hyperplasia, eosinophilia, basophilia, and t(1;8;10)( (p31;q24;q11.2).

Myeloid and lymphoid neoplasms with eosinophilia (M/Ls-Eo) encompass heterogeneous but aggressive hematopoietic disorders triggered by fusion genes or mutations that typically lead to constitutive overexpression of tyrosine kinase. The occurrence of T-lymphoblastic lymphoma in the setting of M/Ls-Eo has been reported rarely in the literature. Herein, we present an unusual case of a 28-year-old male patient who presented with massive lymphadenopathy and T-lymphoblastic lymphoma in the lymph node occurring concurrently with myeloid hyperplasia, eosinophilia and basophilia in peripheral blood and bone marrow biopsy. The syndrome was associated with a novel complex karyotype involving der(8)t(1;8;10)(p31;q24;q11.2). The FISH study was negative for BCR::ABL1, JAK2, PDGFRA, PDGFRB, and FGFR1 rearrangements. The patient's clinical course was aggressive and resistant to multiple lines of intensive chemotherapy regimens. Therefore, he underwent allogenic stem cell transplantation with a fully matched donor. A brief review of the occurrence of T-LBL in conjunction with M/Ls-Eo neoplasm was made with a special focus on molecular aspects.

Acute disseminated candidiasis due to Candida tropicalis with skin and muscular lesions in a patient with Tcell acute lymphocytic leukemia (T-ALL).

Candida tropicalis is the Candida species that is mostly involved in case of acute disseminated candidiasis. We report here a case with whole body dissemination (pulmonary, cutaneous, muscular, hepatic, spinal and cerebral) highlighted by impressive imagery obtained by positron emission tomography scanner in a patient treated for T cell acute lymphocytic leukemia.

Pediatric sudden unexpected death due to undiagnosed mediastinal T-cell lymphoblastic lymphoma: A series of three cases.

The literature on pediatric sudden unexpected death (SUD) due to unrecognized mediastinal neoplasms is limited to a small number of case reports with several cases confirmed to be secondary to T-cell lymphoblastic lymphoma (T-cell LBL). Mediastinal T-cell LBL can be rapidly progressive and potentially fatal due to the compression and obstruction of the airway and/or the great vessels. The clinical presentation is nonspecific with a predominance of respiratory symptoms that are more apparent when the patient is supine. We presented three cases of pediatric SUD attributed to forensic autopsy-diagnosed anterior mediastinal T- cell LBL. Case 1 involved a 2-year-old girl who presented with 9 days of cough and dyspnea. Postmortem examination revealed a firm rubbery mass surrounding the heart and compressing the bronchi. Case 2 involved a 3-year-old girl who suffered from a respiratory tract infection over several days. Autopsy revealed a firm nodular mass compressing the superior vena cava. Case 3 involved a 2-year-old boy who was found unresponsive, lying prone in his crib. He had cold-like symptoms for several days before his death. Postmortem examination revealed a firm, rubbery anterior mediastinal neoplasm surrounding the superior vena cava and great arteries. These three cases demonstrate the importance of identifying children with mediastinal masses that could potentially lead to life-threatening presentations and pediatric SUD. The forensic pathologist should consider a hematologic neoplasm at the time of autopsy in a previously healthy child who dies suddenly.

Pseudohyperkalemia accompanying actual hyperphosphatemia and hypocalcemia in an adolescent with T-lymphoblastic lymphoma.

Tumor lysis syndrome (TLS) is a life-threatening condition that may occur in patients with lymphoma, leukemia, or cancers with high cellular burdens. Without appropriate treatment, electrolyte imbalances, namely hyperkalemia, hyperphosphatemia, and hypocalcemia, can be fatal in patients with TLS. In pseudohyperkalemia, concurrent hyperphosphatemia and hypocalcemia can render devising a treatment strategy challenging. We report an adolescent with T-lymphoblastic lymphoma who presented with pseudohyperkalemia but actual hyperphosphatemia and hypocalcemia, to highlight the importance of accurate clinical interpretations of laboratory data in patients with TLS.

Epidural blockade for alternative management of priapism: A case report of child with T-cell acute lymphoblastic leukemia.

Priapism is a clinical condition that rarely presents with leukemia in childhood. Management of priapism treatment can become more complex and difficult when accompanied by acute leukemia. We presented a 16 years old child with t-cell acute lymphoblastic leukemia (ALL) who developed priapism. Due to the failure of conservative methods and intracavernosal drainage, we performed epidural blockade which has limited data reported with successful results in the literature before shunt surgery.

Characteristics of Central Nervous System (CNS) Involvement in Children With Non-Hodgkin's Lymphoma (NHL) and the Diagnostic Value of CSF Flow Cytometry in CNS Positive Disease.

OBJECTIVE: To investigate the characteristics of central nervous system (CNS) involvement in children with non-Hodgkin's lymphoma (NHL) and the value of flow cytometry (FC) in the diagnosis of CNS disease in pediatric NHL. METHODS: The data of 56 newly diagnosed pediatric NHL patients with CNS involvement (CNS+/mass, CNS+/palsy, CNS+/CSF) were analyzed. The proportions and formats of CNS disease in different pathological types were compared. In addition, FC and conventional cytology (CC) of cerebrospinal fluid (CSF) were carried out in 383 newly diagnosed NHL cases. RESULTS: A total of 383 children with NHL were enrolled. Among these patients, 56 (14.6%) were diagnosed with positive CNS involvement (CNS+), 33 had bulky disease (tumor diameter >10 cm), 32 had bone marrow invasion, 32 had lactate dehydrogenase levels >1000 U/L, and 25 had invasion of more than 4 organs at the time of diagnosis. There were 14 patients with T lymphoblastic lymphoma (T-LBL), 9 with B lymphoblastic lymphoma (B-LBL), 26 with Burkitt's lymphoma (BL), and 2 with Epstein-Barr virus-positive diffuse large B cell lymphoma (EBV + DLBCL). Among the 56 CNS+ patients, 35 were CSF-positive (CSF+); there were 2 patients who were CSF+ via CC detection and 35 who were CSF+ via FC detection. The difference between CC and FC was statistically significant (P < 0.01). In the T-LBL group, 14 patients were CNS+/CSF, and in the B-LBL group, 8 were CNS+/mass. In the BL group, 22 patients were CNS+/mass and 15 were CNS+/CSF. In the anaplastic large-cell lymphoma group, 5 patients were CNS+/mass. Nine of the 56 CNS+ patients had events. The 2-year overall survival rate was 87% ± 0.046%, and the 2-year event-free survival rate was 76.2% ± 0.07%. CONCLUSION: CNS+ diagnoses were more common in pediatric NHL patients with bulky disease and/or bone marrow involvement and/or involvement of more than 4 organs at the time of diagnosis, and they were also common in the EBV + DLBCL and BL groups. FC of CSF showed important clinical significance in the diagnosis of CNS disease in pediatric NHL patients, and it can be used to significantly improve the CNS+ detection rate.

T cell ALL in a child with Ataxia telangiectasia; diagnosis and management challenges.

Ataxia telangiectasia (A-T) is a rare childhood autosomal recessive neurodegenerative chromosomalin stability disorder. It is characterized by high risk of hematological malignancies with T-cell phenotype being the most common, which can present first before the diagnosis of A-T made. The chromosomalin stability in A-T increases the toxicity to radio-chemotherapeutic agents, creating the treatment modification challenges and the deviation from the optimal management protocols. In this case report, we present a 14-month-old boy diagnosed as T cell -ALL. Based on his early presentation, family history of childhood lymphoma, and high AFP, inherited predisposition was suspected, and genetic testing confirms A-T. This report represents the crucial part of clinical suspicion of A-T in similar cases as well as highlighting the importance of an early A-T diagnosis that prevents toxic death due to the extensive regimen of radio- chemotherapeutic agents. The report summarizes the toxicity and modification challenges during management with literature review for the chemotherapy modification experience in such cases.

Intracranial Hemorrhage Associated With T-Cell Acute Lymphoblastic Leukemia With Hyperleukocytosis: A Case Report.

Acute leukemia in children may present with hyperleukocytosis. Symptomatic hyperleukocytosis is a medical emergency that necessitates rapid stabilization of the patient and prompt lowering of the leukocyte count. We report on a patient with intracranial hemorrhage associated with T-cell acute lymphoblastic leukemia with hyperleukocytosis, which is a rare occurrence. A 16-year-old boy with hyperleukocytosis (total white cell count; 398×103/µL) underwent repeated leukapheresis and received supportive treatment until a definite diagnosis of T-cell acute lymphoblastic leukemia was made and chemotherapy was started at 10% of the usual dose. On day 2 of treatment, he had headache, vomiting, and was agitated. Brain magnetic resonance imaging showed bilateral extensive hemispheric and cerebellar punctate areas of hemorrhage and perilesional edema. Chemotherapy intensified to a maximum dose on day 3. If supportive care for tumor lysis syndrome can be promptly provided, initial chemotherapy regimen can immediately be begun at an optimal dose.

Pseudohyperkalemia and Pseudohyponatremia in Two Children with T-Cell Acute Lymphoblastic Leukemia.

Pseudohyperkalemia and pseudohyponatremia are phenomena in which hematologic disorders with high cell counts result in factitious electrolyte measurements that can result in inappropriate treatment. We describe 2 children with leukemia presenting with both disturbances to highlight the importance of correlating electrolyte results from plasma with those from whole blood before intervening.

Retrospective Diagnosis of Ataxia-Telangiectasia in an Adolescent Patient With a Remote History of T-Cell Leukemia.

Ataxia-telangiectasia (A-T) is a rare autosomal recessive disorder characterized by progressive cerebellar degeneration that is typically diagnosed in early childhood. A-T is associated with a predisposition to malignancies, particularly lymphoid tumors in childhood and early adulthood. An adolescent girl with minimal neurologic symptoms was diagnosed with A-T 8 years after completing therapy for T-cell acute lymphoblastic leukemia, following a diagnosis of ATM-mutated breast cancer in her mother. We highlight the importance of recognizing ATM mutations in T-cell acute lymphoblastic leukemia, appreciating the phenotypic heterogeneity of A-T, and defining optimal cancer screening in A-T patients.

T-Cell Acute Lymphoblastic Leukemia in a Young Adult With Thrombocytopenia-absent Radius Syndrome: A Case Report and Review of the Literature.

Thrombocytopenia-absent radius (TAR) syndrome is a rare inherited bone marrow failure syndrome not generally associated with acute leukemia. The authors report a case of T-cell acute lymphoblastic leukemia in an adult female individual newly diagnosed with TAR syndrome. A 347-kb microdeletion of chromosome 1q21.1 involving the RBM8A gene was detected within a gain of whole chromosome 1. Next-generation sequencing on fibroblasts confirmed germline heterozygous deletion of RBM8A but on the other allele, noncoding low-frequency regulatory single-nucleotide polymorphism of RBM8A (rs139428292; rs201779890) were not found. The tolerance of the treatment was unusual and mostly marked by a slow hematopoietic recovery leading to a 6-month delay at the beginning of the maintenance phase. Only 5 cases of acute leukemia were reported in patients with TAR syndrome in the literature: 4 acute myeloid leukemia and one B-cell acute lymphoblastic leukemia. This is the first report of T-cell acute lymphoid leukemia occurring in the context of TAR syndrome.