Triple terapia inmunosupresora con el empleo del Tymogam® en la aplasia medular idiopática / Immunosuppressive triple-therapy using Tymogam® in idiopathic aplastic anemia

Introducción: la terapia inmunosupresora con globulina antitimocítica o antilinfocítica y ciclosporina A se considera el tratamiento estándar para pacientes con aplasia medular muy severa o severa, que no tienen posibilidades de trasplante de progenitores hematopoyéticos. Objetivo: evaluar la respuesta del tratamiento inmunosupresor con el empleo de Thymogam® en pacientes adultos con aplasia medular idiopática. Método: se realizó un estudio descriptivo de 26 pacientes mayores de 18 años con diagnóstico de aplasia medular idiopática, atendidos en el Instituto de Hematología e Inmunología entre enero del 2000 y abril del 2012, que recibieron como parte del tratamiento inmunosupresor, la globulina antitimocítica (Thymogam®). Resultados: se clasificaron14 pacientes como aplasia medular muy severa y 12 como severa. La media de edad fue de 36 años (rango 18 - 74 años). En los primeros 6 meses después de iniciado el tratamiento, el 73 por ciento alcanzó algún tipo de respuesta y de ellos, en 15 (57,7 por ciento) la respuesta fue completa, la que fue más frecuente en los menores de 25 años. El porcentaje de recaídas fue del 26,3 por ciento. Las reacciones adversas más frecuentes fueron fiebre, hipertensión arterial, temblores y sudoraciones. Las principales complicaciones fueron las hemorragias graves y la sepsis. Un paciente desarrolló una leucemia mieloide aguda. La sobrevida global a los 5 años fue del 73 por ciento. Conclusiones: el Thymogam® es una opción terapéutica viable en el tratamiento inmunosupresor de pacientes con aplasia medular idiopática severa y muy severa(AU)

Introduction: immunosuppressive treatment with antithymocyte or antilymphocyte globulin and cyclosporin A is considered the standard treatment for patients with very severe or severe idiopathic aplastic anemia who do not have the possibility of progenitor hematopoietic transplantation. Objective: to evaluate the responses to immunosuppressive treatment using Thymogam® in adult patients with idiopathic aplastic anemia. Methods: A descriptive study was performed in 26 patients older than 18 years with diagnosis of idiopathic aplastic anemia, attended at the Institute of Hematology and Immunology from January 2000 to April 2012, who received antithymocyte globulin (Thymogam®) as part of immunosuppressive treatment. Results: patients were classified as very severe (14) and severe ( 12). The median age was 36 years old (range 18 - 74 years). In the first six months after the start of treatment, 73 percent of patients obtained some kind of response and 15 of them (57,6 percent) had a complete response, which was more frequent in the group of patients with less of 25 years of age. The percentage of relapse was 26,3 percent. The more frequent adverse reactions were fever, arterial hypertension, tremors and sweatiness. The main adverse reactions were severe bleeding and infections. One patient developed acute myeloid leukemia. Overall survival at 5 years was 73 percent. Conclusion: thymogam® is one therapeutic option in the immunosuppressive treatment in patients with very severe or severe idiopathic aplastic anemia(AU)

Marrow hypoplasia: a rare complication of untreated Grave’s disease / Hipoplasia de medula óssea: uma rara complicação da doença de Graves não tratada

Atypical presentation forms of hyperthyroidism are always a challenge to the clinician. We present a female patient with the typical symptoms of thyrotoxicosis, without any thionamides treatment before, associated with pancytopenia, which recovered after euthyroidism state was achieved. Although the major cases of pancytopenia in Grave’s disease are seen as a complication of antithyroid drugs (thioamides), in this case report the alteration in blood tests was associated with untreated hyperthyroidism. In the literature review, we found 19 case reports between 1981 to 2012, but it has been related to a hypercellular bone marrow with periferic destruction. Our case, however, is about a hypocellular bone marrow without fibrosis or fat tissue replacement, which proceeded with a periferic improvement following thyroid treatment. Although rare, pancytopenia, when present, may develop as an unusual and severe manifestation in untreated subjects.

Formas atípicas de apresentação do hipertireoidismo são sempre um desafio para o clínico. Apresentamos uma paciente do sexo feminino, com sintomas típicos de tireotoxicose associado a um quadro de pancitopenia sem nenhum tratamento prévio com tionamidas. A melhora da alteração hematológica ocorreu após recuperação do eutireoidismo. Embora a maioria dos casos de pancitopenia na doença de Graves seja uma complicação das drogas antitireoidianas (tionamidas), neste caso a alteração hematológica foi associada ao quadro de hipertireoidismo não tratado. Após uma revisão na literatura, encontramos 19 relatos de caso descritos no período de 1981 a 2012, nos quais o quadro de pancitopenia estava relacionado à hipercelularidade medular com destruição periférica das células sanguíneas. Nosso caso, entretanto, trata-se de uma pancitopenia com medula óssea hipocelular, sem infiltração por tecido adiposo ou fibrose, que evoluiu com melhora dos elementos do sangue periférico após tratamento do hipertireoidismo. Embora rara, a pancitopenia, quando presente, pode se manifestar como uma severa manifestação se não tratada a condição desencadeadora.

Linfoma marginal del bazo asociado a acidosis láctica tipo B: Caso clínico / Type B lactic acidosis associated with marginal lymphoma of the spleen: Report of one case

Lactic acidosis in the absence of hypoxia or tissue hypoperfusion (type B) is very rare and is associated with the use of some drugs or malignancy. We report a 79-year-old woman, with a marginal non-Hodgkin's lymphoma of the spleen that was subjected to a splenectomy one year ago. She presented with unexplained tachypnea associated with pancytopenia and elevation of IgM to 10 times over the higher normal limit. Laboratory tests showed the presence of metabolic acidosis and high lactic acid levels in the absence of infection, tissue hypoxia or hypoperfusion. She was treated with sodium bicarbonate and steroids without obtaining a reduction in lactate levels. Twelve days after admission, a single dose of Rituximab quickly normalized lactate concentrations and platelet count. After the fourth dose of Rituximab, pancytopenia disappeared and IgM fell to 25% of its baseline concentration.

Subacute combined spinal cord degeneration and pancytopenia secondary to severe vitamin B12 deficiency / Degenarión combinada subaguda medular y pancitopenia secundaria a déficit severo de vitamina B12

CONTEXT: Decreased vitamin B12 concentration does not usually result in clinical or hematological abnormalities. Subacute combined spinal cord degeneration and pancytopenia are two serious and rarely displayed consequences that appear in severe deficits. CASE REPORT: We present the case of a patient with subacute combined spinal cord degeneration and pancytopenia secondary to severe and sustained vitamin B12 deficiency. Such cases are rare nowadays and have potentially fatal consequences. CONCLUSIONS: Vitamin B12 deficiency should be taken into consideration in the differential diagnosis in cases of blood disorders or severe neurological symptoms. Early diagnosis and treatment can avoid irreversible consequences.

CONTEXTO: El descenso de la concentración de vitamina B12 no suele derivar en alteraciones clínicas o hematológicas. La degeneración combinada subaguda medular y la pancitopenia son dos consecuencias graves y poco frecuentes que aparecen en los déficit severos. CASO CLÍNICO: Presentamos el caso de un paciente con una degeneración combinada subaguda medular y pancitopenia secundarios a un déficit severo y mantenido de vitamina B12. Un caso poco común en nuestros días y con consecuencias potencialmente fatales. CONCLUSIONES: Debemos considerar el déficit de vitamina B12 dentro del diagnóstico diferencial en caso de alteraciones hematológicas o síntomas neurológicos graves. Su diagnóstico y tratamento precoz pueden evitar secuelas irreversibles.

Fulminant pancytopenia due to cytomegalovirus infection in an immunocompetent adult

A case of severe and irreversible pancytopenia secondary to acute primary cytomegalovirus infection in an immunocompetent woman is described. The patient presented with thrombocytopenia, lymphopenia, anemia, and abnormal liver function tests. Treatment with corticosteroids and intravenous immunoglobulin was ineffective in reconstituting hemopoiesis. The patient developed severe sepsis and eventually expired.

Megaloblastic anemia patient admitted with pancytopenia and walking difficulty with normal vitamin B12 and MCV levels

Vitamin B12 deficiency may cause hematologic, gastrointestinal, psychiatric and neurological symptoms. Sub-acute combined degeneration of spinal cord, which develops in the deficiency of vitamin B12, may be reversible in case of early diagnosis and treatment. We describe the management of a 50-years old female who got admitted with pancytopenia and elevated LDH, with walking difficulties since last 15 days. B12 and folic acid levels were found in normal ranges. Megaloblastic changes were observed in the bone marrow examination. Abnormal hyperintence signal changes were observed in T2-weighed cervical spinal cord Magnetic Resonance Imaging in posterior row. Due to the high homocysteine level, treatment with parenteral B12 vitamin was initiated. Following the 3-months treatment, hematologic counts and neurological symptoms of the patient were found to be completely recovered at the control visit. Vitamin B12 deficiency should be considered for the patients with pancytopenia, elevated LDH levels and neurological symptoms, even if vitamin B12 and MCV levels are in normal ranges. Vitamin B12 deficiency should be confirmed with the additional assays, such as, the assessment of serum homocysteine and methylmalonic acid levels, and the treatment should be started promptly

Macrophage Activation Syndrome in Juvenile Rheumatoid Arthritis Successfully Treated with Cyclosporine A: A Case Report

Macrophage activation syndrome (MAS) is one of the serious complications of juvenile rheumatoid arthritis (JRA) and recently, cyclosporine A has been found to be effective in patients with corticosteroid-resistant MAS. A 29-yr-old male was admitted with high fever and jaundice for one month. He was diagnosed as juvenile arthritis 16 yr ago. Physical and laboratory results showed hepatosplenomegaly, high fever, pancytopenia and impaired liver and renal function tests, elevated triglyceride and serum ferritin levels. Bone marrow biopsy showed hyperplasia of histiocytes with active hemophagocytosis. He was diagnosed as MAS associated with juvenile rheumatoid arthritis and managed with high-dose corticosteroids initially, but clinical symptoms and laboratory findings did not improve immediately. Finally, he completely recovered after treatment with cyclosporine A (3 mg/kg/day).

Acute lupus hemophagocytic syndrome: report of a case and review of the literature.

The authors reported a case of systemic lupus erythematosus (SLE) with an unusual presentation. The patient presented with acute febrile illness along with progressive pancytopenia related to increasing hemophagocytic activity of histiocytes in the bone marrow. Concomitant polyarthritis, myositis, nephritis, high titer of antinuclear factor (1:2,560) and positive test for anti-DNA antibody made him fit the diagnostic criteria of SLE. No definite evidence of associated infections was confirmed by bacteriologic, serologic and viral studies. He did not respond to empiric antibiotic therapy but dramatically responded to corticosteroid treatment. Therefore, diagnosis of acute lupus hemophagocytic syndrome was made. The clinical presentation, laboratory diagnosis, and management of the patient are discussed and the literature was reviewed and presented.

A case of severe leptospirosis with pancytopaenia

Pancytopaenia is a rare clinical presentation of severe leptospirosis. We would like to report a case of severe leptospirosis that progressed to pancytopaenia despite initial penicillin therapy. The patient needed a second course of antibiotic with doxycycline to improve his persistent symptoms and cytopaenia. Persistent pancytopaenia in severe leptospirosis and its management were reviewed.

Macroglobulinemia de Waldenstron / Waldestrom's macroglobulinemia

Se presenta el caso clínico de un hombre de 70 años que consultó luego de varios meses de mareos, cansancio muscular, baja de peso y palidez. El estudio realizado demostró: pancitopenia con anemia normocítica, normocrómica, arregenerativa; hiperproteínemia con un componente monoclonal; gammapatía monoclonal IgM kappa con cadena liviana lambda libre; ausencia de lesiones osteolíticas; médula ósea con infiltración linfomatosa y disminución de las series eritroide, mieloide y megacariocítica pero sólo con 3 por ciento de plasmocitos; hiperviscosidad sanguínea. Luego de dos ciclos de melfalan y prednisona el paciente reingresa con un síndrome febril y compromiso de conciencia y fallece a los pocas horas con una sepsis a gram negativos (Escherichia coli) y una infección meníngea