Extramedullary diseases as presenting features of aleukemic acute monocytic leukemia]

We present a case of acute monocytic leukemia presenting with extramedullary disease clinically resembling lymphoma. A 36 year-old man presented with arthralgia and was found to have skin eruption, nasopharyngeal mass, hepatosplenomegaly and superficial lymphadenopathy. The biopsies of those lesions as well as bone marrow revealed infiltration of CD45 positive large atypical cells and were interpreted as malignant lymphoma. At that time blood film showed leuko-erythroblastic picture, but no atypical cells were noted. He was treated with CHOP followed by VEPA chemotherapy and achieved partial remission. Four months later headache and double vision occurred with the same atypical cell in the cerebrospinal fluid. The cells were identified as monoblasts by cytochemistry and immunophenotype. The diagnosis of aleukemic acute monocytic leukemia was made and its CNS involvement was successfully treated with chemotherapy with DHAP and intrathecal MTX. However, two months later, blasts finally appeared in the peripheral blood and the patient died of multiorgan failure eight months after presentation. The blasts were positive for T cell markers (CD2, 4, 8) and NK marker (NKH-1) as well as monocytic markers. This finding, together with those by other investigators, may indicate the association between these immunophenotypes and extramedullary manifestations of acute monocytic leukemia.     

Spontaneous complete remission in a patient with acute monocytic leukemia

A spontaneous complete remission was observed in a 47-year-old female with acute monocytic leukemia. Resolution of all abnormalities, including systemic papule, thrombocytopenia, increased numbers of immature monocytoid cells in the peripheral blood and bone marrow, elevation of serum lysozyme and LDH, and trisomy 8 on chromosome analysis, occurred without any treatment. Moreover, the remission was not associated with any infection or blood transfusion, and is persistent for 12-month duration.     

Alkaline phosphatase-positive leukemic monocytes in a child with acute monocytic leukemia

A subpopulation with alkaline phosphatase activity and neutrophilic granules was found in leukemic monocytes from a child with acute monocytic leukemia (M5B). Almost all leukemic cells were strongly positive for nonspecific esterase and phagocytized opsonized zymosans. These findings suggest that the subpopulation are chimeric cells with characteristics of both monocytic and neutrophilic cell lines. The leukemogenesis in this patient might involve the simultaneous expression of differentiation genes otherwise restricted to either cell lineage.     

Recurrent hepatocellular carcinoma after spontaneous regression

The prognosis of untreated hepatocellular carcinoma (HCC) is usually grave, although there have been a few case reports of spontaneous regression. Tumor recurrence after spontaneous regression has been rarely reported, and there have been no previous reports of recurrent hepatocellular carcinoma after spontaneous regression. Two cases of hepatocellular carcinoma that spontaneously regressed and subsequently recurred are presented. The patients' tumors spontaneously regressed, as reflected by the radiologically evidenced reduction in tumor size and markedly decreased alpha-fetoprotein levels. Subsequent tumor recurrences after regression were evidenced by radiologic findings of newly growing nodules and appreci-ably increased alpha-fetoprotein levels. To the authors' knowledge, these two patients represent the first reported cases of hepatocellular carcinoma recurrence after spontaneous regression. In both of these patients, new hepatocellular carcinomas recurred at different sites before the spontaneous regression of the original HCCs was complete. Although the precise causes of the regression and the subsequent recurrence remain to be further investigated, this phenomenon suggests that multicentric hepatocarcinogenicity and intratumoral events in each hepatocellular carcinoma nodule may be involved. Received: July 8, 1999 / Accepted: November 26, 1999     

Mononucleosis syndrome and acute monocytic leukemia

The association of infectious mononucleosis and an immunocompromised host such as occurs in acute leukemia is reported. The most common cause of infectious mononucleosis is Epstein-Barr virus (EBV) and cytomegalovirus (CMV). Patients with mononucleosis syndrome caused by other agents are rare. We report a case of acute monocytic leukemia (AMoL) who developed varicella zoster virus (VZV) mononucleosis syndrome in the bone marrow recovery phase after myelosuppression due to high-dose cytarabine. Mononuclear leukocytes appearing during the mononucleosis syndrome were very similar to the initial leukemic cells. Varicella zoster virus mononucleosis syndrome was confirmed by delayed herpes zoster rash with dermatomal distribution.     

Successful graft-versus-leukemia effect of second bone marrow transplantation on relapsed leukemia cutis that was refractory to intensive chemotherapy and donor lymphocyte transfusions in a patient with acute monocytic leukemia

We report a patient with acute monocytic leukemia (AMoL; M5) who received a second bone marrow transplantation (BMT) with graft-versus-leukemia (GVL) effect on relapsed leukemia cutis, which had been refractory to intensive chemotherapy and donor lymphocyte transfusions (DLTs). A 21-year-old woman was diagnosed with AMoL and achieved complete remission after intensive chemotherapy. The patient received a nonmanipulated allogeneic BMT from her HLA-identical father. Skin tumors developed in her upper extremities, chest, and thigh 11 months after BMT. Leukemia cutis was confirmed by skin biopsy. There was no evidence of relapse in bone marrow. The patient received several courses of chemotherapy and DLTs for the skin relapse, but the skin tumors persisted. The patient then received a second BMT from the same donor. On day 80, grade II acute graft-versus-host disease developed, and the remaining skin tumors were eradicated on day 98, most probably because of GVL effect.     

The distinctive features of acute monocytic leukemia

Acute monocytic leukemia is an uncommon form of acute leukemia. Distinctive clinical features include gingival hypertrophy, lymphoadenopathy, coagulation disorders, and lysozymuria. Blast cell morphology and cytochemistry are diagnostic. Receptors for the Fc fragment of IgG have been demonstrated on the basis of a few cases. The drug VP 16–213 has been shown to be very effective in treatment of untreated and previously treated patients.