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Primary myeloblastoma of the pineal region.

The first case of a pinealis region tumor of the hematopoietic system without known leukemia (so-called primary myeloblastoma, primary granulocytic sarcoma) is reported. Two weeks after resection, the 3.5-year-old patient suffered from a local recurrence. At this time, a bone marrow infiltration by leukemic blasts was diagnosed for the first time. An aggressive chemotherapy and radiation induced a complete remission, over a 26-month follow-up period. We examined the removed tumor tissue by routine histology methods, immuno- and enzyme histochemistry and electron microscopy. The CSF was studied by conventional microscopy and enzyme cytochemistry. We discuss the epidemiological data of nine previously published cases of primary intracranial myeloblastoma.

Brain Neoplasms↗

[Second marrow transplantation following high dose busulfan, etoposide, and Ara-C after testicular relapse in a patient with AML].

Prognosis of second marrow transplantation after leukemia relapse is usually gloomy. We report a patient with AML who was successfully treated by the second marrow transplant following high dose busulfan, etoposide, and Ara-C for the testicular relapse after the first marrow transplantation. A 24-year-old man was diagnosed as having acute myeloid leukemia (AML) in September, 1988. In December of 1989 when he was in early relapse after his 2nd remission, he received the first allogeneic BMT from his HLA identical brother after high dose busulfan and cyclophosphamide conditioning. His posttransplant course was uneventful and graft versus host disease was not observed. Three months after BMT, he noticed swelling on right testicle. Leukemic cell infiltration was confirmed by aspiration cytology. The testicular relapse was followed by marrow relapse. After successful remission induction chemotherapy, he received 17.5 Gy testicular irradiation and second marrow transplantation using high dose busulfan, etoposide, and Ara-C conditioning. Although his posttransplant period was complicated by severe mucositis, high fever and bronchopneumonia, hematologic recovery was obtained by 3 weeks after the second transplant. He is now continuing in complete remission 18 months after the second BMT. This case report suggests that the combination of high dose busulfan, etoposide, and Ara-C could be a choice as a conditioning regimen for resistant AML relapsing after BMT.

Adult↗

Case report with review of Aeromonas infection.

A 31-year-old woman with A. sobria infection and septicemia was reported. The patient was a known case of ANLL,M4 with incomplete remission following successive courses of chemotherapy. There were abscesses in the subcutaneous tissue of right knee and the liver at necropsy. Leukemic cell infiltration in various organs as well as the aforementioned organs were observed. Reported cases of Aeromonas infection with or without diarrhea were also reviewed. According to our knowledge the present case is the first reported case with A. sobria infection with liver abscess in Thailand.

Adult↗

Optic nerve relapse in a child with common acute lymphoblastic leukemia, treated with systemic anti-CD-20 (rituximab).

We describe a two- year old boy who was diagnosed with pre-B acute lymphoblastic leukemia (ALL). He developed a central nervous system (CNS) relapse with optic nerve involvement. Initially he was treated according to the ALL relapse protocol, including CNS radiotherapy. Despite an initial complete reponse, relapse occurred within six weeks of treatment. The leukemic blast cells were CD-20 positive and he was treated with systemic anti CD-20 therapy (rituximab) with no CNS recurrence over a six-month period. He died due to a CD-20 negative bone marrow relapse. This case illustrates a potential role for rituximab in pediatric CD-20 positive malignancies.

Antibodies, Monoclonal↗

[Successful treatment with drainage of hematoma and chemotherapy in a case of Burkitt leukemia presenting with subdural hematoma].

A 53-year-old male was admitted because of pancytopenia and chronic subdural hematoma. Bone marrow was hypercellular with 97% blasts, which expressed CD10, CD19, CD20, and immunoglobulin mu and gamma chains on the cell surface and had chromosomal abnormalities including t(8 ; 22)(q24 ; q11). The patient was diagnosed as having Burkitt leukemia. Because hemiplegia and disturbance of consciousness developed rapidly, the patient was treated with an emergency drainage operation followed by Hyper-CVAD therapy and achieved a sustained complete remission. Dural infiltration of leukemic cells as well as thrombocytopenia was implicated in the pathogenesis of the subdural hematoma, which contained numerous blasts.

Antineoplastic Combined Chemotherapy Protocols↗

[All-trans retinoic acid-induced erythema nodosum in acute promyelocytic leukemia].

A 24-year-old woman with acute promyelocytic leukemia was treated with all-trans retinoic acid (ATRA) as a remission induction therapy. After pneumonia in the neutropenic period was successfully treated with antibiotic treatment, there was recurrence of high fever alone, followed by the appearance of erythema nodosum with pain in her upper limbs on day 25 of ATRA therapy. Skin biopsy neither revealed infiltration of leukemic cells nor suggested Sweet's syndrome. We considered the eruptions to be associated with ATRA, and prednisolone (30 mg/day for 5 days) was administered. Although the administration of ATRA was continued until complete remission of the leukemia, the erythema nodosum rapidly disappeared following short-term steroid therapy and no recurrence was observed. ATRA-induced erythema nodosum is rare, however it should be recognized as a possible adverse effect in ATRA therapy.

Adult↗

[Ultrasonographic findings in hepatoblastoma].

At de National Institute of Pediatrics in Mexico City, the ultrasonographic findings of hepatoblastomas were analyzed in order to establish the echographic pattern of this malignant liver tumor. The ultrasonic images of patient's livers dating back six years were retrospectively reviewed. The hyperrecognisance of the hepatoblastoma as a solid, poorly limited mass, with or without the presence of calcifications, with the displacement of the portal vein and frequently found in the right lobe, were considered as the most relevant echographic characteristics of the hepatoblastoma. The echographic characteristics found of the hepatoblastoma can aid in the sonographic diagnosis of the disease. The leukemic liver infiltrations, the primary liver lymphoma and the metastasis, due to their hyperrecognisance, can be confused with the diagnosis of hepatoblastoma.

Carcinoma, Hepatocellular↗

Three cases of renal relapse after allogeneic hematopoietic stem cell transplantation for childhood acute lymphoblastic leukemia.

Isolated renal relapse after allogeneic hematopoietic stem cell transplantation (alloHSCT) in children with acute lymphoblastic leukemia (ALL) is a rare condition. Generally, in ALL, the sites most frequently affected by extramedullary relapse are the central nervous system (CNS) and the testicles. Here we report on three young boys with relapsed B-precursor ALL, who underwent alloHSCT from HLA-identical siblings and suffered a histopathologically proven isolated unilateral renal relapse (two patients) or a combined renal and testicular relapse (one patient) 6, 10 and 12 months post alloHSCT. In all patients at the time of relapse bone marrow showed complete remission with complete donor hematopoiesis. They all received total body irradiation with partial shielding of the kidneys as part of their conditioning therapy, such that renal shielding could be an explanation for the observed accumulation of renal relapses. Moreover, during the past few years so called immune privilege has been postulated for frequent relapse sites such as the CNS, the testicles and the anterior chamber of the eye. Impaired accessability of these organs by cytotoxic T-cells (CTLs) with a reduced graft-versus-leukemia (GvL) effect after alloHSCT is based on a number of different molecular and cellular mechanisms. Similar mechanisms have been shown to be effective in the tubulointerstitial space of the kidney, rendering the kidney a potentially immune privileged site. Due to these observations we advocate sufficient treatment of the kidneys during conditioning therapy.

Antineoplastic Combined Chemotherapy Protocols↗

[Polycythemia vera with der(15) and der(20) associated with remarkable neutrophilia].

An autopsy case of polycythemia vera with der(15) and der(20) associated with remarkable neutrophilia was reported. A 87-year-old man was diagnosed as polycythemia vera in August 1987. The red blood cell count was 621 x 10(4)/microliters, Ht 58.5% and the white blood cell count 45,400/microliters with 92% neutrophils. The splenomegaly, increased red blood cell volume and the low erythropoietin level were present. The arterial SaO2 value was above 92%. The chromosome analysis of bone marrow cells revealed 46, XY, -15, -20, +der(15)t(15;?)(q13-15;?), +der(20)t(20;?)(q11;?). The breakpoint in No. 20 was in q11. The remarkable leukocytosis with relative and absolute neutrophilia were observed. Particularly late in the clinical course the white blood cell count was 92,900/microliters with 99% neutrophils. The Ph1 chromosome was negative and the bcr rearrangement was not detected. He died of bronchopneumonia in January 1989. At the autopsy findings neither the marrow fibrosis nor the extramedullary leukemic cell infiltration was noticed.

Aged↗