ABSTRACT
ABSTRACT Objective: To describe the case of a child who presented hemophagocytic lymphohistiocytosis (HLH) associated with acute monocytic leukemia after chemotherapy, with hemophagocytosis caused by leukemic cells. Case description: In a university hospital in Southern Brazil, a 3-year-old female was diagnosed with acute monocytic leukemia with normal karyotype. The chemotherapy regimen was initiated, and she achieved complete remission six months later, relapsing after four months with a complex karyotype involving chromosomes 8p and 16q. The bone marrow showed vacuolated blasts with a monocytic aspect and evidence of hemophagocytosis. The child presented progressive clinical deterioration and died two months after the relapse. Comments: HLH is a rare and aggressive inflammatory condition characterized by cytopenias, hepatosplenomegaly, fever, and hemophagocytosis in the bone marrow, lymph nodes, spleen, and liver. Although rare, malignancy-associated HLH (M-HLH) is fatal. The patient in this case report met five out of the eight established criteria for HLH. The evolution of the patient's karyotype, regardless of the diagnostic profile, seemed secondary to the treatment for acute monocytic leukemia. In this case, the cytogenetic instability might have influenced the abnormal behavior of leukemic cells. This is a rare case of HLH in a child with acute monocytic leukemia.
RESUMO Objetivo: Descrever um caso de um paciente pediátrico que apresentou linfo-histiocitose hemofagocítica (LHH) associada à leucemia monocítica aguda pós-quimioterapia, com hemofagocitose causada pelas próprias células leucêmicas. Descrição do caso: Em um hospital universitário do Sul do Brasil, uma menina de três anos foi diagnosticada com leucemia monocítica aguda com cariótipo normal. Após receber protocolo quimioterápico, atingiu remissão seis meses depois do início do tratamento, recaíndo quatro meses após com um cariótipo complexo envolvendo ambos os cromossomos, 8p e 16q. A medula óssea mostrava-se infiltrada por células blásticas vacuolizadas com aspecto monocítico, com evidências de hemofagocitose. A criança apresentou um declínio clínico progressivo e dois meses após a recaída foi a óbito. Comentários: A LHH é uma condição inflamatória rara e agressiva caracterizada por citopenias, hepatoesplenomegalia, febre e hemofagocitose na medula óssea, linfonodos, baço e fígado. A LHH associada a doenças malignas, embora seja uma condição rara, é potencialmente fatal. A paciente deste caso apresentou cinco dos oito critérios estabelecidos para o diagnóstico de LHH. A evolução do cariótipo do paciente, independentemente do perfil do diagnóstico, pareceu ser secundária ao tratamento da leucemia monocítica aguda, sendo que a instabilidade citogenética pode ter influenciado o comportamento atípico observado nas células leucêmicas. Este é um dos raros casos de LHH em uma criança com leucemia monocítica aguda.
Subject(s)
Humans , Female , Child, Preschool , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Leukemia, Monocytic, Acute/drug therapy , Lymphohistiocytosis, Hemophagocytic/etiology , Brazil , Leukemia, Monocytic, Acute/diagnosis , Leukemia, Monocytic, Acute/genetics , Leukemia, Monocytic, Acute/pathology , Fatal Outcome , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/immunology , Lymphohistiocytosis, Hemophagocytic/pathologyABSTRACT
A forty-three-year-old Thai man presented with acute fever and dyspnea for one week with bilateral patchy infiltration, pancytopenia with monoblast. Bone marrow study was consistent with acute monoblastic leukemia. Lung lesions rapidly progressed to acute respiratory failure, which required intubation. Bronchoscopy with bronchoalveolar lavage revealed monotonous monoblast infiltration. Induction chemotherapy with 7 + 3 regimen was administered to halt the progression of leukemic pulmonary infiltration. Although there was clinical improvement, the chest radiograph developed crescent formation in the right upper lung field. Invasive pulmonary aspergillosis was suspected and successfully treated with antifungal agent. After peripheral blood recovery, bone marrow evaluation was performed and complete remission was established. HLA matching was sent to prepare for hematopoietic stem cell transplantation (HSCT). The literature review showed that the appropriate treatment for the patients with t(10;11)(p12;q23) was HSCT, but there was no data concerning correlation of t(10;11)(p12;q23) and pulmonary infiltration. This may be due to the low incidence of leukemic infiltration of acute leukemia patients, which is 0.48% and 3.06% in acute myeloid leukemia and acute monoblastic leukemia, respectively.
Subject(s)
Adult , Antibiotics, Antineoplastic/therapeutic use , Antifungal Agents/therapeutic use , Antimetabolites, Antineoplastic/therapeutic use , Aspergillosis, Allergic Bronchopulmonary/pathology , Bronchoalveolar Lavage , Cytarabine/therapeutic use , Echinocandins/therapeutic use , Hematopoietic Stem Cell Transplantation , Humans , Idarubicin/therapeutic use , Leukemia, Monocytic, Acute/drug therapy , Lung Neoplasms/drug therapy , Male , Pyrimidines/therapeutic use , Thailand , Triazoles/therapeutic useABSTRACT
Presentamos una leucemia monoblástica aguda con múltiples infiltrados dérmicos específicos iniciales rebeldes a la quimioterapia, hematológicamente eficaz. Insistimos en la necesidad de diagnosticar (impronta) y evaluar la magnitud de los mismos con fines diagnósticos (biopsia), así como en la detección de posibles santuarios cutáneos, para lo cual la inmunomarcación de muramidasa resultó de gran valor