We report on an 18-year-old man who had both acute monoblastic leukemia and Marfan syndrome. A diagnosis of Marfan syndrome was established by those characteristics of arachnodactyly, ectopia lentis, mitral valve prolapse, and mitral regurgitation. Findings on bone marrow examination of the patient showed that most of nucleated cells were monoblasts and immunophenotype of those cells showed CD13+, CD33+, CD56+, and HLA-DR+. To our knowledge, this is the second report of leukemia in Marfan syndrome in the world.
Adolescence ; Biopsy, Needle ; Bone Marrow/pathology ; Diagnosis, Differential ; Echocardiography ; Electrocardiography ; Human ; Leukemia, Monocytic, Acute/diagnosis ; Leukemia, Monocytic, Acute/complications* ; Male ; Marfan Syndrome/diagnosis ; Marfan Syndrome/complications*

Adult ; Bone Marrow Diseases ; complications ; Humans ; Leukemia, Monocytic, Acute ; etiology ; Male ; Necrosis

OBJECTIVETo identify the clinical and pathological features of acute myeloid leukemia with B lymphoproliferative disorders.

METHODSThe characteristics of 3 cases of acute monocytic leukemia with untreated chronic lymphocytic leukemia/monoclonal B-cell lymphocytosis were reported with literatures review.

RESULTSThe patients presented with a history of anemia, bleeding and/or fever. Acute monocytic leukemia was diagnosed by bone marrow morphology, cytochemistry and pathology studies. Immunophenotyping by flow cytometry analysis showed a significant population of absolute B-lymphocyte count of > 5×10(9)/L in a patients, similar to that of chronic lymphocytic leukemia.

CONCLUSIONSThe association of acute monocytic leukemia and untreated chronic lymphocytic leukemia/monoclonal B-cell lymphocytosis was a rare event. The abnormal B lymphocytes was likely to be misdiagnosis. Thus, it was important to combine several kinds of laboratory studies, especially flow cytometry to identify this rare disorder.

Aged ; B-Lymphocytes ; pathology ; Female ; Humans ; Leukemia, Monocytic, Acute ; complications ; diagnosis ; pathology ; Lymphocytosis ; complications ; diagnosis ; pathology ; Middle Aged

Aged, 80 and over ; Exanthema ; etiology ; Female ; Humans ; Leukemia, Monocytic, Acute ; pathology ; Leukemic Infiltration ; complications ; diagnosis ; Skin ; pathology

We report a case of therapy-related acute myeloid leukemia after low-dosed topoisomerase II inhibitor (etoposide) treatment for hemophagocytic lymphohistiocytosis (HLH). A 62-yr-old female patient had previously been treated with a HLH-94 protocol containing a low-dose of etoposide (total dose of 300 mg/m2). Thirty-one months later, the patient was admitted to the hematology department with general weakness and upper respiratory infection symptoms. Peripheral blood smear and bone marrow study revealed acute monocytic leukemia. There was no evidence of myelodysplastic syndrome, and a cytogenetic study showed no chromosomal abnormalities.
Bone Marrow/pathology ; Etoposide/administration & dosage/*adverse effects ; Female ; Humans ; Leukemia, Monocytic, Acute/*chemically induced/*diagnosis/therapy ; Lymphohistiocytosis, Hemophagocytic/complications/*drug therapy ; Middle Aged

We report an autopsy case of congenital monoblastic leukemia that developed in monozygotic twins. The twin presented with progressive hepatosplenomegaly at 4 weeks after birth. One twin died of massive bleeding and hypovolemic shock before the treatment started. At autopsy, the liver was diffusely enlarged and showed a diffuse whitish discoloration except for the subcapsular and perivenular areas. Microscopic examination disclosed infiltration of histiocyte-like atypical cells along the sinusoids and portal areas of the liver. Spleen, lymph nodes and choroid plexus were also infiltrated by the tumor cells. However, bone marrow involvement of the tumor was minimal although multifocal. On immunohistochemical staining, these atypical cells were reactive for CD68 (PGM-1) and lysozyme, suggesting that the tumor cells might have been derived from mono- histiocyte. Cytogenetic study revealed 9;11 translocation, which is frequently associated with acute monoblastic leukemia. To the best of our knowledge, this is the first report of congenital monoblastic leukemia of monozygotic twins in Korea.
*Chromosomes, Human, Pair 11 ; *Chromosomes, Human, Pair 9 ; *Diseases in Twins/genetics ; Fatal Outcome ; Female ; Hepatomegaly/complications/genetics/pathology ; Human ; Infant, Newborn ; Leukemia, Monocytic, Acute/complications/*congenital/genetics/pathology ; Liver/pathology ; Splenomegaly/complications/genetics/pathology ; *Translocation (Genetics) ; *Twins, Monozygotic/genetics