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PubMed1981

Childhood acute leukemia: a search for occult extramedullary disease prior to discontinuation of chemotherapy.

Between January 1978 and September 1979, 29 children with acute leukemia in complete continuous remission for three or more years were examined for evidence of occult extramedullary disease immediately prior to discontinuation of chemotherapy. Bilateral open wedge testicular biopsy demonstrated the presence of bilateral leukemic infiltrates in 2/13 boys. Gallium scans prior to biopsy had identified unilateral infiltration in an enlarged testis in one patient but failed to identify microscopic disease in the opposite testis or in the testes of the second patient. Percutaneous kidney and liver biopsies, pelvic ultrasonography, intravenous pyelogram, skeletal survey, cranial computed axial tomography scan, electroencephalography, and ophthalmologic examinations failed to demonstrate evidence of occult disease. Except for testicular biopsy, this study does not support extensive clinical or invasive procedures to identify extramedullary disease prior to discontinuation of chemotherapy.

Adolescent↗
PubMed1982

Urinary 6-hydroxymethylpterin levels accurately monitor response to chemotherapy in acute myeloblastic leukemia.

In our earlier study it was shown that patients with various types of cancer excrete three- to 20-fold higher levels of urinary 6-hydroxymethylpterin as compared to patients with nonmalignant diseases or normal volunteers. In the present study urinary 6-hydroxymethylpterin levels have been measured as an index of disease status in acute myeloblastic leukemic patients on antileukemic drugs. The daily excretory pterin levels and the values of respective blast counts have been graphically presented for four cases with the chemotherapeutic regimens. Remission of the disease was marked by a rapid drop in the urinary 6-hydroxymethylpterin levels and correlated very well with the percentage of blasts. In another study the percentage of blasts and the urinary 6-hydroxymethylpterin were determined simultaneously in four patients undergoing chemotherapy. The data conclusively show that the simple noninvasive determination of urinary 6-hydroxymethylpterin levels accurately and rapidly reflects the status of the disease under treatment and could conveniently be employed as a suitable index for monitoring the effect of chemotherapy as well as the condition of the residual extramedullary leukemic infiltration.

Antineoplastic Agents↗
PubMed1982

Frequency of early death in children with acute leukemia presenting with hyperleukocytosis.

The relationship of hyperleukocytosis, early death, and intracerebral hemmorahage was determined in 547 pediatric patients with acute leukemia. Hyperleukocytosis occurred in 7.3% of patients with acute leukemia. The early mortality rate was significantly increased in hyperleukocytic patients when compared to the nonhyperleukocytic group. Hyperleukocytic patients with ANLL had a significantly greater risk of early death than nonhyperleukocytic patients with ANLL. The difference in the incidence of early death between hyperleukocytic and nonhyperleukocytic patients with ALL/AUL was not statistically significant. In the hyperleukocytic patients intracerebral hemorrhage was the universal cause of early death but this entity did not occur in any of the nonhyperleukocytic patients. There was no correlation of intracerebral hemorrhage to thrombocytopenia or coagulation abnormalities. Autopsies confirmed intracerebral hemorrhage and leukostasis with leukemic infiltration of the brain tissue.

Acute Disease↗
PubMed1982

Myocardial rupture with cardiac tamponade as a lethal early manifestation of acute myeloblastic leukemia.

A 73-year-old woman presented with a two-week history of increasing lassitude and fever. The hemoglobin level was 152 g/l and the total leucocyte count was 348 X 10(9)/l with 97% blasts. Before the diagnosis of acute leukemia could be confirmed by bone marrow examination, the patient developed shock and died within a few minutes. At autopsy a myocardial rupture of the left lateral wall apparently due to a massive leukemic infiltration was found.

Aged↗
PubMed1984

Treatment of acute myeloid leukemia with a combination of intensive induction chemotherapy, early consolidation, splenectomy and long-term maintenance chemotherapy.

The authors developed a therapeutic regimen in which 33 patients aged 11 to 61 years (mean +/- SE, 35.9 +/- 2.3 years) with acute myeloid leukemia (AML) were given intensive induction chemotherapy with Adriamycin (doxorubicin) (ADM), vincristine (VCR) and cytosine arabinoside (ARA-C). Twenty-nine of these patients (88%) attained a complete remission (CR) after 1, 2, or 3 courses and were then subjected to an early consolidation course of chemotherapy, identical to that for induction. After consolidation, all patients in CR received a long-term continuous maintenance therapy in which 6-mercaptopurine (6-MP) and methotrexate (MTX) were alternated, associated with periodic reinforcements with daunorubicin (DNR) and VCR. Twenty-five of the 29 patients who achieved a CR were splenectomized soon after the consolidation course. Histologic sections of the spleens, liver biopsy specimens, and lymph nodes, stained routinely and with the naphthol AS-D chloroacetate esterase (NCA) method, showed mature granulocytes and a few NCA positive mononuclear cells, but no proved leukemic infiltrates. For the 25 splenectomized patients, the probability of remaining in CR at 36 and 54 months was 75% and 66%, respectively; the probability of survival at 36 and 54 months was 85% and 75%, respectively. Age older than 40 years and evidence of extramedullary involvement at presentation appeared to carry a bad prognosis for disease-free survival.

Acute Disease↗
PubMed1984

Response of chronic myelogenous leukemia patients to COAP-splenectomy. A Southwest Oncology Group study.

Eighty-seven patients from 18 institutions with a confirmed diagnosis of chronic myelogenous leukemia were registered on a Southwest Oncology Group protocol for multiagent induction and single-agent maintenance chemotherapy, with randomization to an immunotherapy arm. Elective surgical splenectomy was performed for 42 patients at the completion of 3 months of induction therapy. Final analysis of the study revealed statistically significant survival advantages were correlated with age, splenectomy, the absence of hepatic leukemic infiltrate at the time of splenectomy, and race.

Adolescent↗
PubMed1985

Odynophagia in patients with malignant disorders.

The differential diagnosis of odynophagia in patients with malignant disorders usually includes esophagitis due to herpes, Candida, or gastroesophageal reflux. Two cases and a review of the literature are presented that illustrate that leukemic infiltration of the esophagus and necrosis of the esophageal mucosa following chemotherapy should be considered in addition to the more commonly recognized causes. Esophagoscopy with biopsy and brushing for fungal stains is essential for the correct diagnosis since the various causes for odynophagia may be clinically and radiographically indistinguishable.

Aged↗
PubMed1986

Treatment of hairy cell leukemia with alpha interferons.

A review of clinical research with alpha interferons in the treatment of hairy cell leukemia is presented. The results of several studies have shown that alpha interferons effectively reduce leukemic infiltrates in the bone marrow and other organs, with attendant resolution of cytopenias. Alpha interferons have shown efficacy in the treatment of patients in all clinical stages of the disease, including those previously untreated, and may become the treatment choice for hairy cell leukemia. Hairy cell leukemia will also provide a useful human clinical model in which some of the fundamental mechanisms of interferon activity can be studied.

Dose-Response Relationship, Drug↗
PubMed1987

Megakaryoblastic transformation of chronic granulocytic leukemia.

Three cases of megakaryoblastic transformation of chronic granulocytic leukemia (CGL) are reported. In each case, the leukemic transformation had morphologic features suggesting megakaryocytic differentiation. This was confirmed by positive immunostaining with a monoclonal antibody (HP1-1D) specific for platelet and megakaryocyte glycoprotein IIb/IIIa antigen, which was expressed by the majority of the leukemic blasts in all three cases. Cases with evidence of multilineage differentiation of the leukemic transformation were excluded. A striking feature in two patients was the manifestation of lytic bone lesions and soft tissue masses at presentation. A biopsy of a lytic bone lesion and soft tissue mass in one patient revealed a megakaryoblastic leukemic infiltrate, which by immunocytochemical staining was positive for the megakaryocytic markers, glycoprotein IIb/IIIa antigen, and Factor VIII (von Willebrand factor) antigen. In contrast to granulocytic sarcomas, the megakaryoblastic sarcoma did not stain cytochemically for chloroacetate esterase. The mean survival after acute transformation was 5.3 months. The three cases of megakaryoblastic transformation represented a significant proportion of all CGL blastic transformation cases (ten cases) evaluated by bone marrow examination in our institution during a 13-month period. Megakaryoblastic transformation of CGL may occur more frequently than has been appreciated, and can present as lytic bone lesions or as soft tissue megakaryoblastic sarcomas.

Adult↗
PubMed1987

Fulminant leukemic polyradiculoneuropathy in a case of B-cell prolymphocytic leukemia. A clinicopathologic report.

A 52 year old man with a 10-month history of B-cell prolymphocytic leukemia (PLL) died of an apparent acute fulminant polyradiculoneuropathy, a condition generally attributed to paraneoplastic complication. The pathologic examination disclosed diffuse leukemic infiltrations of the peripheral nervous system. It is suggested that this particularly aggressive form of B-cell chronic prolymphocytic leukemia presented a constellation of features that promoted the invasion of the peripheral nervous system by way of the bloodstream and may explain the unusual clinical presentation.

B-Lymphocytes↗
PubMed1987

Cerebral vasculitis associated with hairy cell leukemia.

Hairy cell leukemia was diagnosed in a 74-year-old man. He was followed for 5 years when he developed confusion and focal neurological signs. Despite investigation and treatment he died. Postmortem study revealed isolated primary necrotizing vasculitis affecting the cerebral arteries. No leukemic infiltration of the central nervous system was found. There is a recognized association between hairy cell leukemia and generalized necrotizing vasculitis of polyarteritis type, however, this is the first case report of isolated cerebral vasculitis associated with this condition. The importance of excluding infective (mycotic) arteries in this type of case is emphasized.

Aged↗
PubMed2002

Cholecystitis as the presenting manifestation of acute myeloid leukemia: report of a case.

We report a case of acute leukemia, which presented as cholecystitis in a previously healthy middle-aged adult. Leukemic involvement of the gastrointestinal tract is a well-known clinicopathological entity. However, leukemic infiltration of the gall bladder wall is a rare occurrence. It has only been previously described in the setting of relapsed disease and, to our knowledge, has never been reported as the primary manifestation of de novo acute leukemia. The patient in question was treated with standard induction and consolidation chemotherapy and remains in complete remission 19 months after his presentation with symptoms consistent with cholecystitis.

Antineoplastic Agents↗
PubMed2004

Myelodysplastic syndrome with central diabetes insipidus manifesting hypodipsic hypernatremia and dehydration.

Central diabetes insipidus (DI) is a rare but recognized complication of myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) that is caused by leukemic infiltration to the hypothalamo-neurohypophyseal system. In rare patients in whom a wide region of the hypothalamus is involved, central DI results in hypodipsic hypernatremia and dehydration. Typical DI symptoms such as polydipsia, polyuria, and marked thirst are concealed in these cases, because the hypothalamic "thirst center" cannot send thirst stimuli to the cerebral cortex. Herein we describe a patient with MDS developing into AML, who presented with hypodipsic hypernatremia and dehydration. A diagnosis of central DI was made on the ground of a low level of serum anti-diuretic hormone (ADH) despite high serum osmolality. A magnetic resonance imaging study revealed attenuation of a physiological "bright spot" of the neurohypophysis. An induction course chemotherapy including regular-dose cytarabine and daunorubicin produced a rapid improvement of hypernatremia. The bone marrow aspirate after two courses of chemotherapy showed complete remission. At that point, ADH release and the "bright spot" were recovered. In order to correctly diagnose central DI in association with hematological malignancies, we should not overlook this atypical type of DI.

Dehydration↗
PubMed1977

Acute myelogenous leukemia subsequent to therapy for a different neoplasm: clinical features and response to therapy.

The clinical characteristics of 10 patients with acute myelogenous leukemia (AML) which developed subsequently to treatment for another neoplasm are described. This disease appears to differ from "spontaneous" AML in being associated with lesser degrees of leukemic infiltration of the marrow and more frequent chromosomal aberrations. Only one of the nine patients who received chemotherapy attained remission status, and the mean and median survivals from the initiation of chemotherapy were 2.7 months and one month respectively. Nine of the 10 patients died as a result of infection. The refractoriness of this form of AML to chemotherapy was borne out by a review of the literature, which revealed only two remissions in 32 treated patients. The implications for the management of this disease are discussed.

Bone Marrow↗
PubMed1989

Congenital erythroleukemia: a case report with morphological, immunophenotypic, and cytogenetic findings.

We report a lethal case of congenital erythroleukemia presenting on the first day of life with peripheral blast cells and a leukemic infiltrate in the placenta. Although initial bone marrow examination did not fulfill the French-American-British (FAB) cooperative group criteria for acute myelogenous leukemia (AML), including M6, a malignant clone was confirmed by cytogenetic analysis: 49,XX, +8, +19, +21. Evolution to erythroleukemia (M6) occurred over a two-month period. The diagnosis of erythroleukemia was supported by immunophenotyping employing an antibody to glycophorin A. The clinical course was complicated by liver failure of unknown etiology. Comparison to previously reported cases of early childhood erythroleukemia is made.

Female↗
PubMed2002

Role of flow cytometry in the diagnosis of lymphadenopathy in children.

To evaluate the combination of fine-needle aspiration (FNA) and flow cytometric immunophenotyping (FCI) in the diagnosis of lymphadenopathy in children, we reviewed a total of 71 FNA specimens from pediatric patients with persistent lymphadenopathy. Two cases were deemed inadequate. In the remaining 69 cases, 54 (78%) were diagnosed as benign lesions, 9 (13%) as Hodgkin's lymphoma, 4 (6%) as non-Hodgkin's lymphoma or leukemic infiltrate, and 2 as metastatic tumors. Of the 69 cases, 25 cases (38%) were diagnosed based on cytomorphology alone, 30 (43%) by combined cytomorphology and FCI, and 19 (28%) by surgical biopsy. In conclusion, FNA is an easy, safe, and reliable procedure in the diagnosis of lymphadenopathy in children. In difficult cases, FCI can be used to exclude non-Hodgkin's lymphomas.

Adolescent↗
PubMed1981

Proceedings of the tumor board of the Children's Hospital of Philadelphia. Testicular leukemia: incidence and management results.

The patient was an eight-year-old black male who presented to the Children's Hospital of Philadelphia (CHP) in June 1977 with foot pain. Abnormal findings on physical examination were diffuse shotty lymphadenopathy without hepatosplenomegaly. Examination of the extremities was normal. There was no evidence of increased bruising or bleeding. Laboratory data revealed a hemoglobin of 11.2 gm/dl, white blood cell count of 26,200/cu mm, and platelet count of 21,000/cu mm. Serum uric acid level was 4.2 mg/dl. The remainder of the laboratory findings were within normal limits. Bone marrow examination revealed a hypercellular marrow replaced with lymphoblasts. Immunologic evaluation showed these cells to have no surface immunoglobin and no rosette formation with sheep red blood cells. The patient received vincristine 1.5 mg/M2 daily, oral prednisone at 40 mg/M2 daily, L-asparaginase 6,000 IU/M2 for nine intramuscular doses, and methotrexate 12 mg intrathecally. After 28 days, bone marrow aspiration showed that the leukemia was in remission. He then received 2,400 rad cranial irradiation over three weeks, along with four more doses of intrathecal methotrexate, given once weekly. Maintenance consisted of monthly pulses of vincristine (1.5 mg/M2), prednisone (40 mg/M2 for five days), daily oral 6-mercaptopurine (75 mg/M2), and weekly oral methotrexate (20 mg/M2). After six months of maintenance, the patient was given a scheduled course of reinduction therapy with vincristine, prednisone, and L-asparaginase. Eleven months after diagnosis both testes were noted to be enlarged on physical examination. Wedge biopsy of both testes revealed leukemic infiltration. Examination of the bone marrow and cerebrospinal fluid (CSF) at that time were unremarkable. The patient was treated with vincristine, prednisone, and L-asparaginase again for four weeks and received intrathecal methotrexate as central-nervous-system (CNS) prophylaxis. Twenty-four hundred rad were given to both testes at 200 rad/day with decrease in testicular size. Maintenance consisted of monthly pulses of vincristine and prednisone with oral methotrexate and 6-mercaptopurine. Seven months after his testicular relapse the patient had a bone marrow relapse. He expired eight months later with disseminated leukemia.

Adolescent↗