RESUMEN
We herein report a rare case of acute basophilic leukemia with t(16;21)(p11;q22) generating the FUS-ERG fusion gene. The basophilic nature of leukemia blasts was demonstrated by cytomorphology, toluidine blue metachromasia, mature basophil-associated antigen expression, and characteristic granules under electron microscopy. The molecular link between t(16;21)/FUS-ERG and basophilic differentiation remains unclear.
RESUMEN
Among 42 consecutive patients with malignant lymphoma who underwent high-dose chemotherapy (HDC) followed by autologous hematopoietic stem cell transplantation (AHSCT), 5 developed hemolytic anemia with fragmented red blood cells (HA-FrRBCs) on days 87-125 (median 107) of AHSCT. Nadir Hb levels ranged between 5.0 and 6.4 g/dL with 2.2-5.6% FrRBCs. All patients developed grade ≥3 hypoxia and heart failure, and 4 developed grade ≥3 hypertension. The ejection fraction of the left ventricle assessed by echocardiography was significantly reduced in 3 patients. Peak creatinine levels were >4 times above the baseline and estimated glomerular filtration rates were reduced to <30 mL/min/1.73 m2. One patient received plasma exchange, while the remaining 4 responded to treatment with diuretics and cardiovascular agents. Hematological parameters normalized within a median duration of 91 days after the development of HA-FrRBCs. Renal and cardiac functions gradually improved, even though renal function did not return to the baseline. HA-FrRBCs associated with cardiac and renal impairments may represent a thrombotic microangiopathy syndrome and are a delayed complication of HDC/AHSCT. The close monitoring of laboratory abnormalities and persistent treatment with cardiovascular agents and diuretics are the mainstay for the management of this condition.
Asunto(s)
Anemia Hemolítica/etiología , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Eritrocitos/metabolismo , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Linfoma/terapia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Creatinina/sangre , Supervivencia sin Enfermedad , Ecocardiografía , Eritrocitos/citología , Femenino , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Hemoglobinas/análisis , Humanos , Linfoma/mortalidad , Linfoma/patología , Masculino , Persona de Mediana Edad , Insuficiencia Renal/etiología , Trasplante Autólogo , Resultado del TratamientoRESUMEN
Transplantation therapy for hematopoietic diseases is conducted by a team led by an attending physician. After transplantation, various adverse effects occur, such as graft versus host disease (GVHD) and thrombotic microangiopathy (TMA), recurrence, and engraftment failure. Therefore, attending physicians are busy with the evaluation of laboratory data, monitoring of immunosuppressants, use of blood products, and management of infectious diseases. Under such circumstances, laboratory technicians play various roles. Our blood test technicians conduct tests to predict outcomes desired by attending physicians and report them quickly and accurately. For that purpose, they must develop skills to interpret test results and examine morphological findings. In this report, we introduce our efforts to improve post-transplantation medical care. [Review].
Asunto(s)
Trasplante de Médula Ósea , Pruebas Hematológicas , Personal de Laboratorio Clínico , Enfermedad Injerto contra Huésped , Enfermedades Hematológicas , Humanos , Inmunosupresores , Microangiopatías TrombóticasRESUMEN
Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL) may include the lymphoid blast crisis of chronic myeloid leukemia (CML-BC). We applied fluorescence in situ hybridization (FISH) of the BCR-ABL fusion gene to peripheral blood and/or bone marrow smears to determine whether the fusion was restricted to mononuclear cell nuclei or if segmented cell nuclei representing mature neutrophils also carried the fusion (Seg-FISH). Among 20 patients with Ph+ ALL without a prior diagnosis of CML, 9 were Seg-FISH+ and 11 were Seg-FISH-. Seg-FISH+ cases were characterized by a higher rate of p210-type BCR-ABL transcripts, higher white cell and blast counts, and a higher rate of myeloid and T-lymphoid antigen expression than Seg-FISH- cases, in addition to 'major route' cytogenetic abnormalities associated with CML-BC. Eighteen patients were treated with tyrosine kinase inhibitors (TKIs) either alone or in combination with multiagent chemotherapy, and 7 underwent allogeneic hematopoietic stem cell transplantation. Progression-free and overall survivals were greater in the Seg-FISH+ group than in the Seg-FISH- group. These results suggest that the Seg-FISH+ group represents lymphoid CML-BC that occurs de novo, while the Seg-FISH- represents Ph+ ALL in the strict sense, and the two groups are associated with survival when treated with TKIs or TKI-combined therapy.