Case 281: Thoracic Air Leak Syndrome in a Patient with Hematopoietic Stem Cell Transplantation and Graft-versus-Host Disease.

HistoryAn 18-year-old man was diagnosed with precursor B-cell lymphoblastic leukemia and underwent transplantation of hematopoietic stem cells from his human leukocyte antigen-matched sister 1 year prior to admission. He was admitted to evaluate progressive shortness of breath and dry cough of 1-month duration. He did not report fever, night sweats, or hemoptysis. Physical examination revealed he was afebrile and had normal pulse oxygen saturation. The examination revealed crepitation on palpation of the anterior neck, expiratory wheezes, and crackles heard at auscultation of bases of both lungs. Extensive maculopapular rash on the skin was consistent with graft-versus-host disease (GVHD). Laboratory tests revealed elevated liver transaminase and bilirubin levels that were attributed to liver GVHD. Nonenhanced thin-section CT of the chest was performed.

Lymphoblastic Lymphoma Involving Multiple Vertebrae.

Acute lymphoblastic lymphoma is a malignant hematologic disease in childhood but rarely initially involves epidural compartment in adults. A 20-year-old male presented with progressive osphyalgia with constipation. Contrasted magnetic resonance imaging showed multiple vertebrae of hypointense T1 signals and an intraspinal epidural lesion. Subtotal resection was achieved. Histopathology suggested malignant B-cell lymphoma with Ki-67 of 90% and positivity of leukocyte common antigen. A bone marrow biopsy was unequivocally diagnostic of B-cell acute lymphoblastic lymphoma followed by chemotherapy (methotrexate) and partial recovery was observed. The marrow biopsy was necessary if without hypercalcemia and abnormal peripheral blood examination.

Thyroid abscess in case of Pre B acute lymphoblastic leukaemia: a rare presentation.

Thyroid abscess is a very rare clinical condition. It usually occurs in immunocompromised individuals or those with underlying malignancy. We report a case of multiple thyroid abscesses in the patient with Pre B acute lymphoblastic leukaemia which developed secondary to hematogenous spread from pyomyositis of right calf muscle. The patient developed sepsis-associated disseminated intravascular coagulation, which got resolved after thyroidectomy. He became afebrile after surgical intervention. Unfortunately, all the cultures were negative. Since there are few case series and reports, there are no clear guidelines for management of thyroid abscess. We conclude that though rare, thyroid abscess may be the cause of persistent fever in immunocompromised patients.

Primary B-lymphoblastic lymphoma of gallbladder involving mandibular bone.

We report the case of a 75-year-old man who presented for evaluation of painless hematuria persisting for more than 1 month. At the time of presentation, the patient did not report any systemic symptoms and had no fever, weight loss, or dysuria. Computed tomography showed several enhancing, sessile polyps in the gall bladder (1.5 cm or smaller). There was no associated stone or biliary dilation. Since no other abnormality was evident, we performed laparoscopic cholecystectomy. He was diagnosed as having B-cell lymphoblastic lymphoma (B-LBL) after surgical resection of the gall bladder (GB). As the left mandibular swelling was developed after the diagnosis of the B-LBL involving GB, facial magnetic resonance imaging (MRI) was added to the imaging scan. Facial MRI revealed mass formation in the left mandible, left medial pterygoid, masticator, and buccinator muscles. The biopsy samples from the mandibular bone were also diagnosed as B-LBL. The definitive pathological diagnosis was B-LBL, stage IV. Systemic chemotherapy was done with subsequent response in size of the left mandible mass.

Acute cerebral infarct with elevated factor VIII level during the thrombocytopenic stage after hematopoietic stem cell transplant.

Thromboembolism is a complication of hematopoietic stem cell transplant. However, a literature search showed no previous reports of cerebral infarction during the thrombocytopenic stage after hematopoietic stem cell transplant. A 35-year-old woman with acute lymphoblastic leukemia (precursor B-cell type) was treated with hematopoietic stem cell transplant after induction and consolidation chemotherapy. On day 2 after transplant, she was unconscious, and had urinary incontinence and left hemiplegia. A computed tomography scan of the brain showed an acute ischemic infarct in the right middle cerebral artery region and an old infarct at the left thalamus without atherosclerosis. Factor VIII level was elevated (190%; normal range, 60% to 150%). She was treated with rehabilitation and low-dose aspirin. At 6 months after transplant, the leukemia remained in remission and she had no further thromboembolic events. This case suggests that prudent treatment of patients who have hematopoietic stem cell transplant may include monitoring for thromboembolism and testing factor VIII level before transplant.

Fatal outcome of rhino-orbital-cerebral mucormycosis due to bilateral internal carotid occlusion in a child after hematopoietic stem cell transplantation.

Rhino-orbito-cerebral mucormycosis is a rare fulminant opportunistic fungal infection that particularly occurs in immunocompromised patients. We present a case of fatal invasive rhino-orbito-cerebral mucormycosis complicated by bilateral thrombotic occlusion of the internal carotid artery with consequent cerebral infarction in a 5-year-old boy after hematopoietic stem cell transplantation for acute pre-B-cell lymphoblastic leukemia.

Low-dose cytarabine and aclarubicin combined with granulocyte colony-stimulating factor for the treatment of relapsed or primary refractory acute lymphocytic leukemia: a retrospective study of 25 Chinese patients.

Despite improvements in treatment, the prognosis of relapsed or primary refractory acute lymphocytic leukemia (ALL) remains poor, and outcomes are worse in older adults with the short first complete remission (CR). Attainment of the second CR by salvage therapy would improve the survival of these patients and may enable them to undergo curative treatment with allogeneic hematopoietic stem cell transplantation. The fact that there are diverse salvage protocols for these adult patients but without a striking CR-induction efficacy indicates that efforts are still needed to indentify new effective reinduction regimens. In this study, the CAG regimen (cytarabine, 10 mg/m(2) subcutaneously every 12 h on days 1-14; aclarubicin, 5-7 mg/m(2) intravenously daily on days 1-8; and concurrent granulocyte colony-stimulating factor, 200 µg/m(2) /day subcutaneously) was administered to 25 patients with relapsed or refractory ALL, including 11 T-cell ALL (T-ALL) and 14 B-cell (B-ALL) patients (age range, 11-61 years; median age, 26 years), to assess its efficacy as a salvage therapy. One course of the CAG regimen resulted in an overall response [CR or partial remission (PR)] rate of 64%, a CR rate of 56% and generally mild adverse effects. An overall response was observed in all 11 T-ALL patients (10 CR and 1 PR) and 35.7% of B-ALL patients (p = 0.0009). The significant treatment potential of CAG regimen for relapsed or primary refractory ALL, especially for T-ALL patients, described in this report would prepare them for a second CR to pursue longer survival.

Necrotizing fasciitis caused by Serratia marcescens after venous access port implantation in a child with acute lymphoblastic leukemia.

Necrotizing fasciitis is a potentially life-threatening infection of deep skin layers and subcutaneous tissues that can easily spread across the fascia plate and is usually the result of a combined infection with anaerobic and aerobic microorganisms. The patient typically complains of excruciating pain, which is not necessarily in accordance with clinical signs. Early recognition of the condition is very important, and aggressive treatment with a combination of antibiotics and surgical procedure is crucial. We present a case of a 15-year-old girl with acute lymphoblastic leukemia who developed necrotizing fasciitis after venous access port implantation during induction chemotherapy.

Primary cutaneous precursor B-cell lymphoblastic lymphoma with late dissemination.

A 20-year-old woman presented with multiple painless nodular swellings on the skin of the extremities and face, without any systemic symptoms. Biopsy with immunohistochemistry revealed a diagnosis of precursor B-cell lymphoblastic lymphoma. There was no extracutaneous site of involvement. The patient denied chemotherapy and was subsequently lost to follow-up. She presented with symptomatic disseminated disease 18 months later and rapidly succumbed to her illness.

[26 year-old male patient with bone marrow transplantation. Report of a partial autopsy]. / Paciente de 26 años trasplantado de médula ósea. Reporte de autopsia parcial.

26 year-old male patient with diagnosis of acute lymphoblastic leukemia in 2006, who underwent chemotherapy and suffered a relapse and pulmonary aspergillosis as a complication. In 2009, he received bone marrow transplant. After it, he developed cutaneous and intestinal graft versus host disease (GVH). He was admitted for diarrhea. Then he presented grade IV dyspnea, patchy alveolar infiltrates on chest computed tomography and pancytopenia with impaired renal function as laboratory findings. He entered Intensive Care Unit, dying 7 days later. The oncologist who discussed the case defined this patient as a high risk case because of type of transplant received, relapse and complications. His diagnostic hypotheses were: CMV infection, pulmonary aspergillosis reactivation, chronic GVH, Pneumocystis jiroveci infection, mycobacteriosis and pseudomembranous colitis. Partial autopsy revealed diffuse intra-alveolar hemorrhage, diffuse alveolar damage, right pulmonary infarction with microthrombosis and bronchiolitis obliterans organizing pneumonia.

Long-term extracorporeal photochemotherapy in a pediatric patient with refractory sclerodermatous chronic graft-versus-host disease.

Sclerodermatous chronic graft-versus-host disease (cGVHD) following allogeneic hematopoietic stem cell transplantation (HSCT) in children is difficult to treat and life-threatening. Extracorporeal photochemotherapy (ECP; photopheresis), an immunomodulatory therapy that involves the infusion of autologous peripheral blood leukocytes after ex vivo exposure to the photoactive agent 8-methoxypsoralen and ultraviolet A radiation, is an effective treatment for steroid-refractory cGVHD. After undergoing allogeneic HSCT for pre-B-cell acute lymphoblastic leukemia, a 14-year-old boy developed extensive sclerodermatous cGVHD that was refractory to prednisone, tacrolimus, and sirolimus. ECP was administered over the course of 53 months, during which the skin softened substantially and immunosuppressive therapy was discontinued. This case suggests that long-term ECP is a viable option in children with sclerodermatous cGVHD.

Primary precursor B cell lymphoblastic lymphoma of uterine corpus: case report and review of the literature.

INTRODUCTION: Primary lymphomas of the female genital tract are rare. Most involve the cervix rather than the uterine corpus. Many cases of primary endometrial lymphoma are diagnosed as diffuse large B cell type, whereas the precursor B cell lymphoblastic type is extremely rare. MATERIALS AND METHODS: We report a case of precursor B cell lymphoblastic lymphoma of uterine corpus which was successfully treated with surgery and chemotherapy. Staging evaluation revealed tumor limited to the uterine corpus (stage I(E)). After receiving a total abdominal hysterectomy, bilateral salpingooophorectomy, lymph node dissection and combination chemotherapy, the patient is currently free of disease after follow-up of 42 months. CONCLUSION: If correct diagnosis is established and appropriate therapy is chosen, the prognosis of precursor B-LBL of uterine corpus is expected to be good. The literature on primary precursor B cell lymphoblastic lymphoma of the uterine corpus is reviewed.

Isolated ovarian relapse of pre-B acute lymphoblastic leukemia: a case report.

BACKGROUND: Acute lymphoblastic leukemia is a malignant disease of the bone marrow in which early lymphoid precursors proliferate and replace normal marrow hematopoietic cells, resulting in a marked decrease in the production of normal blood cells. CASE REPORT: We report a case of isolated ovarian relapse 7 years after the primary diagnosis in a patient, who was seemingly in clinical remission following unilateral ovariectomy and second-line chemotherapy. CONCLUSION: In contrast to testicular relapse, ovarian relapses in acute lymphoblastic leukemia are rarely reported. Surgical removal of the mass followed by chemotherapy is the therapeutic standard.

Relapse of acute lymphoblastic leukemia in the jaw.

This case report describes the clinical case of relapse of precursor B-cell acute lymphoblastic leukemia in the jaw of a 19-year-old female patient who presented with facial swelling, sensory disturbances of the face, and teeth mobility 10 months after a successful allogenic bone marrow transplant. The oral and dental presentations were the only features indicating leukemic relapse in this patient.