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PubMed1983

Lymphoma and leukemia manifested by steroid-responsive polyneuropathy.

Subacute polyneuropathy that responded to prednisone was the initial symptom in two patients, one of whom was later found to have histiocytic lymphoma and the other, chronic lymphocytic leukemia. Sural nerve biopsy specimens in both showed extensive segmental demyelination. In the first patient, there was invasion of the myelinated axons by macrophage processes, and the later course of the neuropathy appeared to parallel that of the lymphoma. In the second patient, there was diffuse lymphocytic infiltration of the perineurium and endoneurium, lymphocytes were found beneath the basal lamina, and the demyelination was characterized by extensive vesicular degeneration of the myelin sheath. The demyelination appeared to be cell mediated in the first patient. This was probably the mechanism in the second patient as well, but simple mechanical compression by infiltrating leukemic cells was another possible explanation.

Chronic Disease↗
PubMed1994

Mononeuritis multiplex. A harbinger of acute leukemia in relapse.

OBJECTIVE: To report the findings in a patient who developed severe mononeuritis multiplex in the setting of hematologic remission from acute myeloid leukemia. DESIGN: Single case report of the patient, hospital course, and autopsy findings. PATIENT: A 63-year-old woman with a history of acute myeloid leukemia in hematologic remission experienced a succession of acute clinical neuropathies (left median, right radial, and left sciatica) several months before hematologic relapse of leukemia. Electrophysiologic tests localized the abnormalities of the left median and right radial nerves to the arms, and a magnetic resonance imaging scan of the right arm revealed focal swelling of the radial nerve proximal to the elbow. The patient refused treatment for leukemic relapse and died about 6 months after the onset of the neuropathies. An autopsy revealed leukemic infiltrates in multiple nerves without appreciable involvement of the cauda equina or many of the proximal nerves. CONCLUSION: Mononeuritis multiplex, without polyradiculopathy or diffuse peripheral neuropathy, can be a presenting feature of leukemia.

Female↗
PubMed1989

Ophthalmic manifestations of leukemia.

We prospectively examined the eyes of 120 patients with leukemia. Only 38% of patients had normal results of examination. Leukemic infiltrates were present in 3% of patients, other findings related to leukemia were seen in 39% of patients, and 20% of patients had unrelated abnormalities. Visual loss was seen in at least 5% of the patients in this study.

Adolescent↗
PubMed1992

Fine-needle aspiration biopsy of the iris.

Fine-needle aspiration biopsy (FNAB) specimens obtained from nine consecutive iris lesions were examined. The lesions included primary malignant melanoma (four cases), metastatic melanoma, metastatic adenocarcinoma, leukemic infiltrate, lymphocytic infiltrate, and epithelial ingrowth. Subsequent histopathologic correlation was performed in all cases. Patient treatment influenced by the results of the FNABs included enucleation (three cases), clinical observation (two cases), external beam irradiation (two cases), resection, and radioactive plaque application. No complications occurred from the FNABs. Fine-needle aspiration biopsy of the iris can be performed with local anesthesia at the slit lamp as an outpatient procedure. In general, FNAB is a safe, effective method of obtaining diagnostic material from primary neoplastic, secondary neoplastic, and degenerative processes involving the iris. Limitations of the procedure include discrepancies in interpretation of the cytologic study and inadequate specimen.

Adult↗
PubMed1976

Histiocytic medullary reticulosis in acute lymphocytic leukemia of T cell origin.

A patient with acute lymphocytic leukemia of T (thymic-derived) cell origin was successfully treated and was maintained in remission for four months by combined chemotherapy. He died following a seven-week, fulminant course with fever, refractory pancytopenia, and marked hepatosplenomegaly. The autopsy showed lymphoid leukemic infiltration and extensive histiocytic medullary reticulosis in various organs. The possible relations between these two lymphoreticular diseases are discussed.

Antineoplastic Agents↗
PubMed1980

Juvenile chronic granulocytic leukemia: emphasis on cutaneous manifestations and underlying neurofibromatosis.

Five patients with juvenile chronic granulocytic leukemia are described. Two patients had multiple cafe-au-lait spots compatible with von Recklinghausen's neurofibromatosis, and three had cutaneous xanthomata. Recurrent cutaneous leukemic infiltrates were noted in two patients. The clinical course of all five patients was characterized by recurrent respiratory symptoms and pulmonary infiltrates that responded to antileukemic therapy in three. Chemotherapy controlled the symptoms but did not influence the eventually fatal outcome.

Child↗
PubMed1986

The value of skin biopsies in febrile, neutropenic, immunocompromised children.

We assessed the value of 41 skin biopsy specimens obtained from 32 immunocompromised patients with fever and neutropenia. Fifty-six percent (23/41) of these biopsy specimens resulted in a specific diagnosis. These diagnoses included infection in 12 cases, graft-vs-host disease in nine, leukemic infiltrate in one, and drug reaction in one. The remaining 18 biopsy specimens showed either nonspecific changes or normal tissue. The infectious agents identified included Aspergillus in nine patients, Candida in three patients, and Staphylococcus aureus in one patient. As a result of the biopsy findings, therapy was altered in 49% (20/41) of the cases. The most frequent therapeutic change was initiation of amphotericin B for a demonstrated fungal infection. We found skin biopsies to be a valuable diagnostic tool in the assessment of the febrile, neutropenic, immunocompromised patient with skin lesions.

Adolescent↗
PubMed1994

Fine needle aspiration in the evaluation of children with lymphadenopathy.

OBJECTIVE: To evaluate fine needle aspiration of enlarged lymph nodes in children. DESIGN: Retrospective study of children who were referred for fine needle aspiration of a palpable lymph node that was of concern to their clinicians. SETTING: Large, urban, tertiary care pediatric hospital. SUBJECTS: One hundred nineteen patients (123 cases) aged 10 months through 21 years during the 30-month period from January 1990 to June 1992. RESULTS: Aspirated material in 13 cases (10.6%) was deemed inadequate for cytologic diagnosis. In the remaining 110 cases, five aspirates revealed malignancy: two patients had Hodgkin's disease, one had non-Hodgkin's lymphoma, one had a leukemic infiltrate, and one had rhabdomyosarcoma. The findings in two cases were suspicious for malignancy, but the lymph nodes were found to be benign on excisional biopsy. No false-negative findings for malignancy were detected. The conditions of patients in the remaining 103 cases were diagnosed as a benign process, most commonly reactive lymphadenitis. Of 57 aspirates cultured, eight (14%) were positive for a microorganism. No significant complications were encountered in any of the 123 cases. CONCLUSION: Fine needle aspiration of enlarged lymph nodes in children is a safe, reliable (accuracy, 98%) procedure that often obviates the need for an excisional biopsy.

Adolescent↗
PubMed1999

Pulmonary hypertension in patients with myelofibrosis secondary to myeloproliferative diseases.

We examined the clinical characteristics of six patients with myelofibrosis secondary to myeloproliferative diseases whose clinical courses were complicated by pulmonary hypertension to determine possible causal links between the two disorders. Six patients (four male, two female), with diagnoses of myeloproliferative disease, myelofibrosis (one with polycythemia vera, three with agnogenic myeloid metaplasia, one with unclassified myeloproliferative syndrome, one with essential thrombocytosis), and pulmonary hypertension are presented. Measurement of the pulmonary artery pressure was performed by Doppler echocardiography in all patients and by right sided heart catheterization in four patients. The range of resting pulmonary artery systolic pressure was 35 to 47 mmHg above the mean right atrium by echocardiography. One patient had autopsy evidence of pulmonary myeloid metaplasia and interstitial fibrosis; another had acute leukemic infiltration of the lung parenchyma. All patients had thrombocytosis; symptomatology in one patient with marked thrombocytosis improved with plateletpheresis. Two patients suffered systemic thrombosis. All patients had severe hepatomegaly. Two patients had evidence of left ventricular dysfunction. The interval between the development of dyspnea and death was less than seven months in five of the patients. A causal link between pulmonary hypertension and myelofibrosis secondary to myeloproliferative diseases is suggested for each patient. Hematopoietic infiltration of the pulmonary parenchyma, portal hypertension, thrombocytosis, hypercoagulability, and left ventricular failure may account in part for the development of pulmonary hypertension in these patients. Patients with myelofibrosis and dyspnea should have Doppler echocardiography to evaluate pulmonary artery pressures.

Adult↗
PubMed1997

Abdominal ultrasound findings during and after treatment of childhood acute lymphoblastic leukemia.

BACKGROUND: The treatment of acute leukemia in childhood has been increasingly successful. Concurrently, severe leukemia-related gastrointestinal complications have become more common. METHODS: We evaluated the findings of the abdominal ultrasound (US) examinations of 52 children with acute lymphoblastic leukemia (ALL) who had severe clinical symptoms indicating infection or abdominal complication during chemotherapy treatment or after the cessation of such treatment and assessed the impact of these findings on patients' subsequent treatment and survival. RESULTS: Our study presents ten cases of typhlitis with a prevalence of 9%, all of which were rapidly diagnosed by US and had a favourable outcome. We also found focal intra-abdominal parenchymal lesions in six children, five of them due to fungal infection and one due to leukemic infiltration. Several other intra-abdominal pathologies significant for the patients' treatment are also reported. DISCUSSION: We believe that abdominal US is a useful, rapid, safe, and accurate imaging method for children with ALL suspected to suffer from leukemia- or chemotherapy-related gastrointestinal complications. More invasive imaging methods are seldom needed. CONCLUSIONS: According to our results, abdominal US gives the necessary information in most of the cases and provides prompt diagnosis, which may prevent possible fatal complications.

Abdomen↗
PubMed2000

Increased CA 125 serum levels in patients with advanced acute leukemia with serosal involvement.

BACKGROUND: CA 125 is a tumor marker used for the diagnosis and monitoring of ovarian carcinoma. This marker also has been found to be increased in patients with serosal effusion derived from nonneoplastic inflammatory disease and in a few instances of advanced non-Hodgkin lymphoma with serosal involvement. METHODS: CA 125 levels were tested in the serum of 15 patients with acute myeloblastic leukemia (AML) at the time of diagnosis and in 3 patients with advanced leukemia with serosal involvement. In two patients with elevated serum CA 125 levels, a CA 125 assay was performed on leukemic cells and on the supernatant fluid of short term liquid culture. RESULTS: Increased serum CA 125 was found in the three patients with acute leukemia with extramedullary localization and serosal effusion, whereas it was normal in 15 AML patients tested at the time of diagnosis. CA 125 was not detectable in leukemic cell extracts nor in the supernatant fluid of primary cultures. CONCLUSIONS: These results indicate that leukemic cells were unable to produce CA 125 and suggest that its elevation in the serum is likely due to a serosal inflammatory reaction caused by the leukemic infiltration.

Adult↗
PubMed1997

Fine-needle aspiration cytologic findings in a case of lymph node tumor of plasmacytoid monocytes.

We present a unique case of fine-needle aspiration (FNA) from a lymph node with subsequent histologic diagnosis of tumor of plasmacytoid monocytes (PMs). The patient had an associated myeloproliferative disease which terminated into an acute myelomonocytic leukemia. In a 95% ethanol-fixed, hematoxylin-eosin (H&E)-stained smear, the tumor cells appeared monomorphic, medium size, with oval to indented nuclei, finely stippled chromatin, and small nucleoli; the cytoplasm was scanty and slightly eccentric. The cytologic picture suggested some subtypes of small-cell non-Hodgkin's lymphomas or a leukemic infiltration. In the FNA specimen, the cells appeared immunocytochemically negative for some B- and T-cell markers (MB2, L26, LN1, and UCHL1) and strongly positive to KP1, a known histyocyte-macrophage and myeloid marker. The FNA differentiated diagnoses are discussed.

Biopsy, Needle↗
PubMed1975

Complications and total care of a child with acute leukemia.

The complications which occur in a child with acute leukemia depend on the stage of the disease and the therapeutic regiman. Most children will present with some manifestation of marrow failure. An occasional child will have marked leukocytosis and disturbance of organ function due to massive leukemic infiltrates. Metabolic disturbances such as hyperuricemia and hyperphosphatemia-hypocalcemia may develop, expecially after therapy is initiated. The myelosuppression and immunosuppression due to drug toxicity may result in opportunistic infections. Other toxicities which can occur with a chemotherapeutic regimen are numerois and varied, and the physician must be cognizant of them in order to minimize damage. Therapy to the central nervous system, either for subclinical or clinical disease, has been associated with a variety of symptoms ranging from meningismus to paraplegia and death. To prevent the development of these complications, and to manage them effectively if they occur, the physician must be knowlegeable about their etiology, clinical and laboratory manifestations, and treatment.

Acute Disease↗
PubMed1976

Effects of chemotherapy on the central nervous system. A study of parenteral methotrexate in long-term survivors of leukemia and lymphoma in childhood.

Twenty-three children surviving more than 5 years from the diagnosis of leukemia, lymphoma, or malignant histiocytosis were evaluated for clinical evidence of central nervous system disease. Severe impairment, consisting of seizures, paraplegia, and dementia was present in 4, all of whom received methotrexate (MTX) and other agents for 2 to 7 years. Brain biopsies in 3 of these children showed white-matter gliosis and no evidence of viral or other infections or leukemic infiltrates. Of the remaining children 10/19 were found to have mild clinical or EEG abnormalities. All had received i.v. MTX with other drugs for 2-6 years; 5 did not receive cranial irradiation. Common to all impaired patients was the administration of intravenous methotrexate in relatively high doses over a prolonged period of time. Impairment in nervous system function may present as a spectrum of deficiencies, with the most severe resulting in death, or as in the 4 patients described here, profound dementia and dependence. Less dramatic changes in functioning, may, however, result from various combinations of chemotherapy and radiation therapy. Methods of assessing their etiology and impact on survivors need now to be devised.

Adolescent↗
PubMed1976

Clinico-pathologic correlation of liver damage in patients treated with 6-mercaptopurine and Adriamycin.

Eleven adults with refractory leukemia treated with a combination of 6-mercaptopurine and Adriamycin developed hepatic dysfunction manifested by elevations of serum total bilirubin, alkaline phosphatase, and glutamic oxaloacetic transaminase. Liver tissue obtained at necropsy showed intrahepatic cholestasis (eight cases), hepatocellular necrosis (ten cases), leukemic infiltration (two cases), and fatty change (nine cases). Neither this frequency nor severity of hepatocellular destruction has hitherto been associated with 6-mercaptopurine at the dose levels used in this study, nor has Adriamycin previously been found to be hepatotoxic. It is postulated that Adriamycin potentiated the hepatotoxicity of 6-mercaptopurine in these patients.

Adult↗
PubMed1976

A case of T-cell lymphoma with convoluted lymphocytes.

A case of T-cell lymphoma, as defined by immunologic studies of lymph node, peripheral blood, and cultured cells, is presented. Convoluted lymphocytes were noted in the original lymph node biopsy, in cerebral spinal fluid preparations and terminally, in the peripheral blood. The prominent neuologic abnormalities representing both central and peripheral nervous system involvement were atypical. Other features included skin and testicular infiltration, leukemic transformation, and refractoriness to therapy. A somewhat similar clinical picture has been reported in other patients with diffuse, poorly differentiated lymphocytic lymphomas, some of which have proven to have a T-cell origin. T-cell lymphoma may represent a distinct clinical entity that calls for modifications of our traditional therapeutic approaches.

Adult↗
PubMed1978

Ovarian carcinoma terminating in acute nonlymphocytic leukemia following alkylating agent therapy.

Rapidly fatal acute myelogenous leukemia (AML) occurred in a woman with advanced (Stage III) ovarian carcinoma who was treated with thiotepa for 30 months. This patient was 1 of 10 long term survivors and represented less than 2% of patients with advanced ovarian carcinoma with regional metastases who received long term chemotherapy during the period 1947-1975. Acute leukemia developed 44 months after initial diagnosis and was preceded by a 10 month period of pancytopenia following cessation of thiotepa. The leukemia did not respond to treatment and the patient expired 3 weeks after its onset. At autopsy, leukemic infiltration of organs was seen, but there was no evidence of carcinoma. A review of the literature suggests that the development of AML reported in ovarian cancer patients is related to alkylating agent therapy.

Adult↗
PubMed1980

Radiotherapy (2500 rad) for testicular leukemia: local control and subsequent clinical events: a Southwest Oncology Group study.

The effectiveness of radiotherapy, 2500 rad over two weeks, in treating leukemic infiltrates of the testicles was studied in 38 boys who met the requirements for tissue conformation of testicular involvement and examination of the bone marrow. The study group was heterogeneous with respect to specific histology and prior therapy. Complete regression of testicular infiltrates was confirmed by repeated biopsy examinations of 32 of 33 patients undergoing the procedure. The single treatment failure occurred in a boy with acute myelogenous leukemia. In all other patients, local disease control following radiotherapy persisted throuthout the remainder of the clinical course. Three of 5 children, however, showed evidence of reseeding of the testicle as a part of the relapse process at post-mortem examination. Statistical analysis of data from the 35 patients with acute lymphocytic leukemia showed the subsequent course of the disease with respect to next relapse, involving either bone marrow (BM) or the central nervous system (CNS), to be dependent on the acute leukemia prognostic group, as determined by age and peripheral white blood cell count (WBC) at the time of diagnosis, and timing of extramedullary disease (EMD). Patients with poor prognosis at the time of diagnosis and EMD afterward had a 3.8 times greater risk of a subsequent BM or CNS relapse than did patients with good or average prognosis and no EMD at any time (P = 0.07). Of the candidate prognostic factors examined with repect to survival, only the number of prior BM relapses was of statistical significance (P = 0.044). Children with two or more prior BM relapses had the worst prognosis for survival from testicular relapse, with a death risk of 3.6 times greater per unit of time than that of children with no or one prior BM relapse. Protective BM and CNS rescue therapy was recommended for those otherwise in complete remission (CR) at the time of testicular relapse. The median times to next relapse for patients receiving both BM and CNS recue therapy and for patients given CNS recue only were 42+ and seven weeks, respectively ( P = 0.09). The type of rescue received did not appear to affect survival time following testicular CR.

Adolescent↗