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Diabetes insipidus as a presenting symptom of acute myelogenous leukemia.

This report describes a case of diabetes insipidus associated with acute myelogenous leukemia. An 11-year-old boy presented with fatigue, polydipsia and polyuria. His evaluation revealed a diagnosis of acute myelogenous leukemia FAB-M2, and a water deprivation test confirmed the diagnosis of central diabetes insipidus. His brain magnetic resonance imaging (MRI) showed a thickened, enhancing pituitary stalk with absence of the normal hyperintense signal in the posterior pituitary. He was treated with systemic chemotherapy, intensive intrathecal therapy, and 1,000 cGy to the pituitary. The patient achieved a remission but continued to need desmopressin therapy to control his diabetes insipidus. Diabetes insipidus is a rare complication of acute myelogenous leukemia that can be caused by leukemic infiltration of the pituitary. The diabetes insipidus is irreversible despite intensive systemic and central nervous system chemotherapy and radiation.

Child↗

Congenital acute myelogenous leukemia presenting as palpable renal masses in a neonate.

Our report concerns a neonate who was noted to have palpable kidneys soon after birth. Her clinical and radiologic findings were compatible with the diagnosis of autosomal recessive polycystic kidney disease. The patient eventually died of sepsis-related multiorgan failure, and on autopsy, surprisingly, was found to have leukemic infiltrates in the kidney. Healthcare providers need to be aware that congenital acute myelogenous leukemia, although rare, can present in the newborn period with nephromegaly.

Autopsy↗

Iris and anterior chamber involvement in acute lymphoblastic leukemia.

The authors describe a 6-year-old child, with a history of acute lymphoblastic leukemia, who presented with leukemic infiltration of the anterior segment of one eye mimicking anterior uveitis. After tissue diagnosis, administration of radiation therapy to the affected eye resulted in dramatic improvement of the condition. Involvement of the iris and anterior chamber is a recognized but unusual manifestation of leukemia. In the absence of systemic relapse, local radiation therapy without chemotherapy is highly effective, although the prognosis in these patients may be guarded.

Child↗

Aleukemic congenital leukemia cutis.

A newborn girl had typical "blueberry muffin" skin lesions, which showed histopathologic features of myelomonocytic leukemia cutis. We could not demonstrate leukemic infiltration of bone marrow in four aspirates. Her course was complicated with primary pulmonary hypertension, which led to death at 7 months of age. We emphasize the persistence of skin lesions in the absence of bone marrow infiltration by leukemia throughout the course of the disease.

Bone Marrow Examination↗

DNA-analysis of human testicular samples by cytofluorometry.

Germ cells in human testicular samples have been estimated by histology and quantitated by flow cytometry. There is an excellent relationship between the two techniques. These samples were classified histologically as "normal", "reduced number of germ cells" or "no germ cells". A definition of "normalcy" as determined by flow cytometry as having at least 38% of the cells in the haploid state, is proposed. Testicular samples from prepubertal boys with infiltrating leukemic cells often show an increase in the percentage of S-phase cells indicating the presence of tumor. These results indicate the value of flow cytometry in the identification of reproductive and neoplastic disorders.

Adolescent↗

Adult T-cell leukemia with generalized cytomegalic inclusion disease and pneumocystis carinii pneumonia.

An autopsy case of adult T-cell leukemia with generalized cytomegalic inclusion disease and pneumocystis carinii pneumonia is reported. Tumor cells had T-cell characteristics (E-rosette) and cerebriform nucleus similar to Sèzary cells. Generalized lymphadenopathy, hepatosplenomegaly and an ectopic pancreas in the ileum were found at the time of autopsy. Histologically, leukemic infiltration was observed in almost every organ, and perivascular infiltration, vascular invasion were conspicuous findings. Cytomegalic inclusion bodies were observed in most organs (lungs, salivary glands, pancreas, liver, ectopic pancreas, sweat gland, stomach, thyroid gland, pituitary body, etc.). An acute hepatitis, probably caused by cytomegalovirus, was also noted. Presumed correlation of adult T-cell leukemia, cutaneous T-cell lymphoma and T-cell lymphoma was discussed.

Adult↗

Spontaneous rupture of the spleen in acute myeloid leukemia.

The results of an autopsy of an 80-year-old Japanese male with acute myeloid leukemia who died of spontaneous rupture of the spleen are reported. The patient was admitted because of anorexia, fatigue, weight loss, and multiple skin eruptions. Hematological examinations indicated a rapid increase in myeloblasts. The patient collapsed on the 28th hospital day, immediately after complaining of severe epigastralgia and vomiting. He died ten hours later. The autopsy revealed extensive leukemic infiltration of the bone marrows, spleen, lymph nodes, skin, and other internal organs. The spleen was enlarged and was ruptured in places at the hilar portion. Massive intraperitoneal hemorrhage from the rupture was the direct cause of death. The mechanisms of splenic rupture are discussed.

Aged↗

Transient abnormal myelopoiesis followed by acute megakaryoblastic leukemia with extramedullary tumors. An autopsied case of Down's syndrome.

An autopsied case of acute megakaryoblastic leukemia is described in a 20 months old, Japanese female infant with Down's syndrome. She presented spontaneous remission of transient abnormal myelopoiesis in her neonatal period, which was followed by acute megakaryoblastic leukemia 1 year later. The clinical picture of acute megakaryoblastic leukemia was initially characterized by peripheral pancytopenia with a few blasts, the absence of hepatosplenomegaly, but ended in overt leukemia characterized by increase of blasts, marked hepatosplenomegaly, and elevated LDH. She died 6 months after the onset at 20 months of age. Autopsy findings revealed widespread leukemic infiltration comprised of megakaryoblasts and megakaryocytes, and extramedullary tumors in the left tibia, the liver, both kidneys, and the endocardium of the heart. Identification of the megakaryocytic cell line was performed in immunohistochemistry and electron microscopy. Chromosomal analyses of peripheral blood disclosed 47, XX, +21, in her neonatal period but disclosed 48, XX, +G, +G, in acute megakaryoblastic leukemia.

Acute Disease↗

Leukemia cutis in blastic transformation of chronic myelocytic leukemia: TdT positive blasts and response to vincristine and prednisone.

A patient with chronic myelocytic leukemia (CML) in blast transformation with extensive involvement of the skin is reported. The leukemic infiltrates consisted of blasts with lymphoblastoid morphology which were positive for terminal deoxynucleotidyl transferase (TdT) by fluorescent assay. The infiltrates rapidly resolved with vincristine and prednisone therapy. Similar recurrence 4 and 8 months later responded as dramatically to the same therapy and to cytosine arabinoside. This unusual case of extensive leukemia cutis supports the evidence that patients in blast crisis with TdT positive cells are responsive to vincristine and prednisone. The implications of this case in terms of the origin of these cells based upon morphology and TdT content, as well as the diagnosis and therapy, are discussed.

Aged↗

Zoledronate-induced remission of acute panmyelosis with myelofibrosis.

Acute panmyelosis with myelofibrosis is a rare and aggressive form of acute myeloid leukemia. We describe a new case with a huge proliferation of megakaryocytes, blast cells and reticulin fibers. The patient was treated with zoledronate, a third-generation bisphosphonate, and a gradual recovery from pancytopenia was observed. A new bone marrow biopsy performed 4 months later showed a surprising disappearance of the leukemic infiltration. Ten months after the diagnosis, the patient is still in healthy condition. This may support the recently described anti-tumor activity of zoledronate.

Acute Disease↗

Isolation of paralysis-inducing murine leukemia viruses from Friend virus passaged in rats.

Four clones of murine leukemia viruses (PVC-111, PVC-211, PVC-321, and PVC-441) were isolated from a paralyzed Fischer rat which had been infected with rat-passaged Friend leukemia virus. PVC-211 and PVC-321 viruses induced hind leg paralysis in rats and killed them within 1 month, and PVC-441 did so within 2 months after infection, whereas PVC-111 did not within 4 months. PVC-321 and PVC-441 but not PVC-111 virus grew well in brain and spinal cord media. The viral antigens were found often in glia cells and rarely in neurons of the rats infected with each of these PVC viruses. All of the PVC viruses induced neuronal degeneration but neither inflammation nor leukemic infiltration in the spinal cord. The isolated viruses were all ecotropic and NB-tropic. Age dependency of the susceptibility of rats to paralysis induction was observed.

Animals↗

Therapeutic effect of a Gag-nuclease fusion protein against retroviral infection in vivo.

Recently, remarkable progress has been made in developing effective combination drug therapies that can control but not cure retroviral replication. Even when effective, these drug regimens are toxic, they require demanding administration schedules, and resistant viruses can emerge. Thus the need for new gene-based therapies continues. In one such approach, capsid-targeted viral inactivation (CTVI), nucleases fused to viral coat proteins are expressed in infected cells and become incorporated during virion assembly. CTVI can eliminate infectious murine retrovirus titer in tissue culture. Here we describe transgenic mice expressing fusions of the Moloney murine leukemia virus (Mo-MuLV) Gag protein to staphylococcal nuclease. This work tests the protective effect and demonstrates in vivo proof-of-principle of CTVI in transgenic mice expressing endogenous proviral copies of Mo-MuLV. The antiviral protein-expressing mice are phenotypically normal, attesting to the lack of toxicity of the fusion protein. The Mo-MuLV infection was much less virulent in transgenic littermates than in nontransgenic littermates. Gag-nuclease expression reduced infectious titers in blood up to 10-fold, decreased splenomegaly and leukemic infiltration, and increased life spans up to 2.5-fold in transgenic relative to nontransgenic infected animals. These results suggest that gene therapies based on similar fusion proteins, designed to attack human immunodeficiency virus or other retroviruses, could provide substantial therapeutic benefits.

Animals↗

Retrograde pancreatic venography: autopsy study.

Changes of the venous system in various pancreatic diseases were evaluated by retrograde pancreatic venographic studies done on 110 autopsy specimens of the pancreas. Fifty-one normal specimens exhibited well filled, regularly arranged veins. Postmortem autolysis (ten specimens) caused penetration of contrast medium into the parenchyma. Acute pancreatitis (six specimens) was associated with hypervascularity and venous irregularity. Chronic pancreatitis was associated with unevenly distributed venous deformities. Pseudocysts caused avascular foci which displaced adjacent vessels. Seven pancreatic cancers produced irregular encasement and occlusion of pancreatic and peripancreatic veins with thrombosis of major portal trunks-changes differing substantially from those caused by other diseases. Periancreatic cancer invading the pancreas caused encasement of superficial and occasionally deep pancreatic vessels. Acute leukemic infiltration demonstrated extreme deformity with complete structural disorder of intrapancreatic branches. Retrograde pancreatic venography, if used clinically, would enable radiographic diagnosis of the most common diseases of the pancrease.

Acute Disease↗

MR imaging of the knee. Part II. Chronic disorders.

Sixty patients with symptoms of chronic disease of the knee joint were evaluated with high-resolution, thin-section magnetic resonance (MR) imaging. MR imaging depicted a wide variety of knee joint abnormalities including osteochondritis dissecans, medullary infarcts, epiphyseal osteonecrosis, intraarticular osteochondral fragments, synovial cysts, joint effusions, intraarticular soft-tissue tumors, synovial disease, leukemic infiltration of bone marrow, Osgood-Schlatter disease, and nonossifying fibroma. In two cases MR imaging depicted bone infarcts not seen on both radionuclide bone scans and standard radiographs. The highly detailed depiction of the articular cartilage was of particular importance in predicting arthroscopic findings in cases of osteochondritis dissecans. In two cases, a soft-tissue mass (pigmented villonodular synovitis) and a large osteochondral fragment undetected at arthroscopy were accurately localized with MR imaging. The results indicate that MR imaging is capable of providing information that might otherwise require multiple, sometimes invasive diagnostic procedures.

Chronic Disease↗

Pleural effusion in chronic myelomonocytic leukemia.

Pleural effusion in 4 patients with chronic myelomonocytic leukemia (CMML) is described in this report. According to the literature, pleural effusion in CMML is a poorly understood and rare occurrence. Two of our patients presented with pleural effusion as an initial symptom while the other 2 developed it during the course of the disease. In only 1 patient was the pleural effusion due to leukemic infiltration while in the other 3 it was a reactive phenomenon. Peripheral lymphadenopathy was observed only in the former patient who died of acute leukemia. After prednisolone therapy the pleural effusions resolved in the other 3 patients.

Aged↗

Primary Intracranial Manifestation of CD7/CD56-Positive Acute Myelogenous Leukemia.

BACKGROUND: CD56 which is considered as a marker of natural killer cells is also expressed in some cases of acute myelogenous leukemia (AML) and is involved in cell adhesion mediating extramedullary leukemic infiltration. CD7/CD56 coexpression has been suggested to be a distinct biological and clinical entity of AML. PATIENT: This is a report of a 53-year-old woman who developed CD7/CD56-positive AML with primary manifestation as intracranial tumor. The patient reported of neurological impairment (impairment of visus and occurrence of double pictures). Cranial computed tomography showed an intracranial tumor, and histological examination exhibited myeloid blast cells. Peripheral leukocyte count at admission was within the normal range (5,32 Gpt/l), and percentage frequency of blasts in the blood smears was 54%. Cytological bone marrow examination showed diffuse infiltration by the same myeloid blast cells. The immunophenotype was CD7/CD13/CD33/CD38/CD56/ HLA-DR-positive. The blast cells were myeloperoxidase-positive but lactoferrin-negative. Thus, diagnosis of acute myeloid leukemia (M2 FAB) was established. Treatment consists of chemotherapy (Ara-C and anthracycline) and local radiation of the intracranial tumor. After treatment patient achieved a complete remission. CONCLUSION: With regard to the literature CD7/CD56-positive AML have a high incidence of central nervous system involvement which should be kept in mind and may be associated to CD56 expression. Copyright 2000 S. Karger GmbH, Freiburg

Journal Article↗

Intracranial lymphoblastic sarcoma mimicking a primary brain tumor.

A lymphoblastic sarcoma mimicked a medulloblastoma in a 15-year old girl with a history of acute lymphoblastic leukemia diagnosed at age of 2 years. There was no evidence of leukemic infiltration in the brain during the 13-year latency period after the original diagnosis. She had received both prophylactic intrathecal methotrexate and central nervous system irradiation. This case emphasizes the need to consider leukemia as the cause of a 'brain tumor' in any patient that has a prior history of leukemia, regardless of the tumor manifestations or the remission duration of the leukemia.

Brain Neoplasms↗

Molecular basis of familial and acquired phagocytosis deficiency involving the tetrapeptide, thr-lys-pro-arg, tuftsin.

The biological activity and metabolism of the phagocytosis stimulating tetrapeptide (Thr-Lys-Pro-Arg) tuftsin, are discussed. Its effect is shown to be highly specific. It stimulates the phagocytic activity of the blood polymorphonuclear leukocyte. A unique familial deficiency of the tetrapeptide is described. In such patients, moderate to severe infections occur at high frequency. These are most pronounced in children. Biochemical and symptomatic evidence can readily be obtained in one or more children. At least one parent of either sex shows clinical signs or laboratory evidence of defective phagocytosis. Another type of deficiency results from removal of the spleen or from loss of specific function due to leukemic infiltration or embolism.

Adolescent↗