Fractura lumbar como presentación de un linfoma linfoblástico de células B / Lumbar fracture as presentation of B-cell lymphoblastic lymphoma

Introducción: El linfoma linfoblástico de precursores de células B (LLB) es responsable del 2% de los casos de los linfomas diagnosticados en la edad pediátrica. El retraso en su diagnóstico es habitual, debido a la poca especificidad de los síntomas asociados. Exponemos un caso clínico con una presentación clínica muy poco común. Caso clínico: Paciente de 14 años de edad que acudió a nuestra consulta por un dolor en la rodilla, la cadera y el muslo izquierdo de 40 días de evolución, que le provocó incapacidad para deambular 1 semana antes de su ingreso. En su abordaje inicial se indicó tratamiento con ketorolaco y piroxicam, lo que propició una mejoría transitoria del dolor. Se realizaron radiografías de la columna lumbar, la cadera y la extremidad inferior, una tomografía computarizada (TC) y una resonancia magnética (RM). Las radiografías detectaron lesiones líticas, que posteriormente fueron documentadas en la TC, en el trocánter y la cabeza del fémur izquierdo y en la primera vértebra lumbar. La RM demostró, además, la presencia de adenomegalias supraclaviculares y una masa paravertebral en la octava vértebra torácica. El aspirado de médula ósea fue negativo para la infiltración, y la biopsia de una de las adenomegalias supraclaviculares reveló un linfoma tipo B. Conclusión: El LLB es una variedad de linfoma unicelular poco común en pediatría, que generalmente se diagnostica en estadios avanzados por su rápido crecimiento (AU)

Introduction: B-cell lymphoblastic lymphoma contributes 2% of the diagnosed lymphomas in the pediatric age. The diagnosis is often delayed by the low specificity of presenting symptoms. We illustrate an uncommon B-cell lymphoblastic lymphoma presentation. Case report: A 14-year old female presented with a 40-day history of left knee, hip and thigh pain that produced intermittent limping, associated with one week history of limping. During her initial medical assistance ketorolac and piroxicam were prescribed, partially decreasing pain. She evolved walking disability, leading her to hospitalization were X-rays, CT scan and MRI were ordered. Osteolithic lesions were found and confirmed by CT scan in 1st lumbar vertebra and left femur head and trochanter. MRI found bilateral supraclavicular lymphadenopathies along a paravertebral tumor at T8 level. Marrow aspiration was negative and biopsy of the supraclavicular masses revealed a B-cell lymphoma, which was further characterized by immunohistochemistry in B-cell lymphoblastic lymphoma. Conclusion: B-cell lymphoblastic lymphoma is a rare tumor in children and is usually diagnosed in advanced stages due to their rapid growth and delay in the diagnosis. The most important diagnostic tool for the general pediatrician is to maintain clinical suspicion due to the low specificity of the clinical symptoms (AU)

Non-myeloablative stem cell transplantation in patients with relapsed acute lymphoblastic leukemia: results of a multicenter study.

Using non-myeloablative conditioning, allogeneic hematopoietic stem cell transplantation (HSCT) was conducted in 43 ALL patients in a CR2. The median age of the patients was 19 years. Patients received oral busulfan 4 mg/kg/day for 2 days; i.v. cyclophosphamide 350 mg/m(2)/day for 3 days; and i.v. fludarabine 30 mg/m(2)/day for 3 days. Oral cyclosporin A 4 mg/kg was started and methotrexate 5 mg/m(2) was delivered on days 1, 3, 5 and 11. The median CD34+ cell dose received was 5.0 x 10(6)/kg. The medium time to achieve a granulocyte count above 0.5 x 10(9)/l was 14 days. Thirteen patients were alive 30-1050 days after the HSCT. The 3-year overall survival rate was 30%. Ten patients (23%) developed acute GVHD, whereas eight patients (18.6%) developed chronic GVHD. Thirty patients died between days 47 and 1050 after the HSCT, most of them (70%) because of an ALL relapse. One hundred-day mortality was 15%, whereas transplant-related mortality was 21%. These results are inferior to those obtained using the same allografting method in other leukemias, probably as a consequence of poor susceptibility to the graft-versus-leukemia effect of the ALL cells beyond first remission as compared with other hematological malignancies.

Non-myeloablative hematopoietic stem cell transplantation is of limited value in advanced or refractory acute myeloblastic leukemia. The Mexican experience.

Allogeneic hematopoietic stem cell transplantation (HSCT) is an effective strategy for preventing relapse of acute myelogenous leukemia (AML). We analyzed the outcome of 31 primary AML patients who received a reduced-intensity conditioning regimen for allogeneic HSCT in first or second remission. Thirty-one AML patients, 20 in first complete remission (FCR), 8 in second complete remission (SCR) and 3 in a partial remission (SPR) were included. All received busulfan 4 mg/kg/d/2 days, fludarabine 30 mg/m(2)/d/3 days and cyclophosphamide 350 mg/m(2)/d/3 days as conditioning regimen. The median number of CD34+ cells infused was 5.6 x 10(6)/kg and 5.2 x 10(6) in FCR and SCR group, respectively. All patients received cyclosporine-A (CsA) and methotrexate as graft vs. host disease (GvHD) prophylaxis. All patients showed myeloid engraftment (neutrophils >0.5 x 10(9)/l) after a median of 13 days in FCR group and 15 days in SCR group. Platelet recovery >20 x 10(9)/l was achieved after a median of 13 days in both groups. Relapse for 20 patients in FCR was 35% compared to 91% for 11 in SCR/SPR (p < 0.05). Conclusions. Reduced-intensity conditioning followed by allogeneic HSCT can induce stable remission in primary AML patients transplanted in FCR. A high relapse rate was documented in patients with refractory or relapsed AML.

Outpatient allografting using non-myeloablative conditioning: the Mexican experience.

A group of 132 patients with both malignant and nonmalignant conditions was allografted using the 'Mexican' method of non-ablative conditioning. The conditioning was delivered on an outpatient basis and the procedure was planned to be conducted on outpatients in all cases. While 103 patients (78%) were able to complete the procedure totally as outpatients, 29 (22%) were hospitalized because of fever, mucositis or acute graft-versus-host disease. In a multivariate analysis, although differences were not statistically significant, it was found that the patients who were allografted as outpatients had higher levels of hemoglobin (12 versus 11.8 g/dl), higher platelet counts (248 versus 191 x 10(9)/l), lower white blood cell counts (11.7 versus 12.4 x 10(9)/l), higher Karnofsky scale scores (100 versus 90%) and lower creatinine levels (0.9 versus 0.93 mg/dl). A total of 86% of the patients with normal values for these variables could be allografted as outpatients, whereas only 67% of those with abnormal values completed the entire procedure as outpatients. It is concluded that allografting can be accomplished totally on an outpatient basis using the 'Mexican' reduced intensity-conditioning regimen.

Results of an outpatient-based stem cell allotransplant program using nonmyeloablative conditioning regimens.

Using nonmyeloablative, immunosuppressive, fludarabine (FLU)-based conditioning regimens, we have performed allogeneic peripheral blood stem cell transplants in 26 patients (8 with chronic myelogenous leukemia, 6 with acute myelogenous leukemia, 10 with acute lymphoblastic leukemia, 1 with myelodysplasia, and 1 with thalassemia major). Conditioning consisted of FLU/busulphan/cyclophosphamide/cyclosporin-A (CyA)/methotrexate, or FLU/melphalan/CyA/methotrexate. The median granulocyte recovery time to 0.5 x 10(9)/l was 11 days, whereas the median platelet recovery time to 20 x 10(9)/l was 12 days. Twelve patients did not need red blood cell transfusions, and 8 did not need platelet transfusions. In 21 individuals (81%), the procedure could be completed fully on an outpatient basis. Follow-up times range between 30 and 600 days: one patient failed to engraft and recovered endogenous hemopoiesis; six out of 26 patients developed acute graft-versus-host disease (GVHD) whereas 7/22 developed chronic GVHD. Twelve patients (46%) have died, nine of them with a relapsing disease and three with GVHD; median post-transplant survival (SV) was 300 days, whereas the 12-month SV was 42%. The 100-day mortality was 3.8% and the transplant-related mortality was 11.5%. This procedure is substantially less costly than its counterpart, using in-hospital myeloablative conditioning regimens, and it may represent another approach in the management of patients requiring an allogeneic stem cell transplant.

Hematopoietic stem cell allografts using a non-myeloablative conditioning regimen can be safely performed on an outpatient basis: report of four cases.

Using a non-myeloablative, immunosuppressive, fludarabine-based conditioning regimen, we performed allogeneic peripheral blood stem cell transplants totally on an outpatient basis in four patients (two with chronic myelogenous leukemia, one with acute myelogenous leukemia and one with thalassemia major). The median granulocyte recovery time to 0.5 x 109/l was 10 days and the lowest absolute neutrophil count was 0.064 x 109/l; only one patient developed thrombocytopenia below 20 x 109/l. No patient required red blood cell transfusions and one was given a single prophylactic platelet transfusion. All patients are alive at 210-390 (median 285) days and have definite evidence of chimerism; one developed biopsy-proven GVHD on day 50, with a limited cutaneous rash. The procedure is less costly than its counterpart using myeloablative conditioning regimens and may represent another approach in the management of patients requiring an allogeneic stem cell transplant. Bone Marrow Transplantation (2000) 25, 131-133.

Bone marrow transplantation in a child with hemophagocytic lymphohistiocytosis using a less toxic conditioning regimen.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare non-neoplastic, frequently fatal disease of childhood. HLA-matched bone marrow transplantation (BMT) can bring about long-term remission and an eventual cure. METHODS: We report on the beneficial effect of BMT in a 2-month-old male using a less intensive conditioning regimen. The regimen included busulfan at 4 mg/kg/day (total dose 16 mg/kg), etoposide at 300 mg/m2/day (total dose 900 mg/m2), and cyclophosphamide at 50 mg/kg/day (total dose 150 mg/kg). Prophylaxis for graft-vs.-host disease included methotrexate and cyclosporine. RESULTS: An absolute neutrophil count of 500 microL was noticed on + day 12 (engraftment day). At present, i.e., 400 days after the procedure, the patient is asymptomatic, his physical examination is normal, and a slightly increased level of gamma-glutamyl-transpeptidase (GGT) and alkaline phosphatase are the only laboratory abnormalities. CONCLUSIONS: In this case, the conditioning regimen was adequate for the eradication of the disease and allowed persistent engraftment without significant toxicity. The results in our patient suggest that a less toxic regimen is feasible and permits rapid engraftment without compromising the effectiveness of chemotherapy.

[Intermittent interferon alfa in the treatment of hairy cell leukemia]. / Interferón alfa intermitente en el tratamiento de la leucemia de células peludas.

OBJECTIVE: To evaluate if short and intermittent courses of human recombinant interferon alpha (IFN) are useful in the long term palliation of hairy cell leukemia. METHODS: Nine patients with hairy cell leukemia received 3 megaunits of IFN thrice a week for 12 weeks. They received 8 weeks of IFN upon relapse or after 10 months of followup every year. RESULTS: A hematological remission was obtained in all cases before 12 weeks. Only three patients, because of relapse, required therapy before 10 months. All the patients are alive and well after a median followup of 62 months. CONCLUSIONS: Short courses of IFN proved to be an alternative in the treatment of hairy cell leukemia. It is less expensive and was effective in the initial therapy and maintenance of selected patients with this kind of leukemia.

Dexamethasone in the treatment of meningeal leukemia.

To determine if dexamethasone has a role in the treatment of meningeal leukemia, 8 consecutive patients with acute lymphoblastic and signs or symptoms of CNS were included in the study. After the confirmation of leukemic blast cells on cerebrospinal fluid, they received intrathecal and IV dexamethasone; 3 days later the patients received "triple" intrathecal chemotherapy with dexamethasone, methotrexate and cytarabine, and the spinal fluid was studied again. All patients had good clinical response and 7 out of the 8 patients showed reduction on the CSF cell count after the use of dexamethasone alone. The results suggest that dexamethasone is a lymphocytic agent that could play a more active role in the prevention and therapy of meningeal leukemia and should be preferred over hydrocortisone in the so called "triple" intrathecal chemotherapy for the prevention and treatment of CNS leukemia.