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This 32-year-old patient with hairy cell leukaemia demonstrated extensive bony involvement in a pattern not previously described. Although the disease continued to progress during interferon treatment, a change to a regime of deoxycoformycin resulted in an excellent response.
Sweet's syndrome (acute febrile neutrophilic dermatosis) is characterized by an acute onset of erythematous plaques, fever, and leukocytosis. This syndrome has been reported to be associated with leukemia including chronic myelogenous leukemia (CML). Sweet's syndrome seen in patients with leukemia is usually associated with active and/or refractory disease. Imatinib Mesylate (STI-571, Gleevec) is widely used for therapy of CML. In this case report, CML cell infiltration of the skin was documented by fluorescence in situ hybridization (FISH) analysis in a patient with chronic-phase CML on Imatinib Mesylate (STI-571, Gleevec), who was at the time in molecular remission.
A 53-year-old man presented with focal jacksonian seizures that were due to cerebral tumour manifestations. Stereotactic biopsy revealed infiltration by lymphocytes compatible with the diagnosis of chronic lymphocytic leukaemia (CLL). Simultaneously, Binet stage II CLL with bone marrow infiltration was diagnosed. Cranial radiotherapy with 36 Gy was performed. One year after diagnosis, the patient is in stable disease. Cerebral involvement is rare but may occur in early CLL. Cranial radiotherapy may lead to lasting remission. In case of spinal fluid involvement intrathecal chemotherapy is recommended.
The polymerase chain reaction (PCR) method was used to determine the presence and amount of human T lymphotropic virus type I (HTLV-I) proviral DNA in central nervous system (CNS) tissue obtained at autopsy from 6 patients with HTLV-I-associated myelopathy (HAM)/tropical spastic paraparesis (TSP), 1 patient with adult T-cell leukemia (ATL) and CNS infiltration of leukemic cells, and 9 control subjects with other neurological disorders. HTLV-I pX and env but not pol DNA were detected in CNS tissue from 5 of 6 patients with HAM/TSP. The ATL samples were positive for pX, env, and pol DNA by PCR. None of the control samples was consistently positive for HTLV-I by PCR, but all showed positive bands on beta-globin PCR. Quantitative PCR combined with histological studies showed no correlation between HTLV-I proviral DNA amounts and extent of perivascular mononuclear cell infiltration in the HAM/TSP CNS. Also, the amounts of pX and probably env DNA were greater in the HAM/TSP samples than in the ATL sample, although the extent of mononuclear cell infiltration was far less in the HAM/TSP samples than in the ATL sample. Therefore, in addition to infiltrating mononuclear cells, constituent cells of the CNS may harbor the HTLV-I genome, at least in the pX and env regions, in patients with HAM/TSP.
Acute febrile neutrophilic dermatosis (AFND, Sweet's syndrome) is clinically characterized by fever, neutrophilic leukocytosis, and tender dermal plaques. Histological examination typically reveals infiltration of the dermis by neutrophils. In three patients (2 female, 1 male, 54-59 years) with acute leukemia (2 myelogenous, 1 lymphoblastic) dermal plaques developed during febrile episodes in chemotherapy-induced pancytopenia. The clinical appearance was compatible with AFND. The diagnosis was substantiated by skin biopsies which showed dense neutrophilic dermal infiltrates without leukemic cells. Leukocytoclastic vasculitis was considered as differential diagnosis. Plasma levels of soluble adhesion molecules ICAM-1, VCAM-1, and E-selectin regulating leukocyte transendothelial migration were in the normal range. Systemic glucocorticoids were avoided because of the high risk of infection during prolonged bone marrow aplasia. The lesions were treated with topical steroids and resolved without scarring within 1-5 weeks. AFND has been reported in association with acute leukemia at normal or elevated white blood cell counts. Although implausible from a pathophysiological point of view, similar neutrophilic dermal infiltrates were found in three patients during chemotherapy-induced pancytopenia with white blood cell counts distinctly below 1 x 10(9)/l.
Large granular lymphocyte leukemia (LGLL) is defined as clonal proliferation of LGLs in peripheral blood. The following studies were conducted to address some issues in chronic LGLL. (1) Chronic LGLL is characterized by the indolent course, and the diagnosis of leukemia is difficult in such patients as those without distinct organomegaly and/or any evidence of monoclonality. We performed immunohistological studies in a patient with persistent NK lymphocytosis. No organomegaly had been seen in the patient during a three-year-observation, who died from cerebrovascular accident. The autopsy findings revealed multi-organ infiltration including spleen, liver, bone marrow, lymph nodes and lung. These findings suggest that the cells of chronic LGLL have infiltrative capacity characteristic of malignant cells. (2) Lymphocytosis in chronic LGLL is usually stable for a long period. We found that both T- and NK-LGLL cells strongly expressed CD95, an apoptosis related protein. Anit-CD95 did not induce apoptosis, but suppressed proliferation induced by IL-2 or anti-CD3. These results suggest that CD95-CD95 ligand system is involved in the slow cell growth characteristic of chronic LGLL. (3) CD4+CD8+ double positive (DP) cases are rarely seen in LGLL, and the physiologic counterpart of the leukemic cells has not been determined yet. We found that the DP-LGLL had alpha alpha type in the CD8 subunit and did not express RAG-1, these findings being characteristic of peripheral T cells. We also found that they expressed IL-4 mRNA and secreted IL-4 on activation. These results strongly suggest that DP-T-LGLL represents an expansion of a rare subset of peripheral DP-T cells, possibly derived from IL-4 activated CD4 single positive T cells.
In the group of 75 ALL patients treated between 1980 and 1989, two women (ages 25 and 17, both FAB-L2 and CALLA +, after 56 and 34 months of continuous CR, respectively) were found to have a relapse manifested as a local infiltration of skin, with involvement of the adjacent lymph nodes in one patient. The leukaemic character of the skin infiltration was confirmed by skin needle aspiration and tissue biopsy expressing CD10 and CD24. Both patients were given intensified systemic chemotherapy and local X-ray irradiation. Complete remission was obtained with the disappearance of the skin infiltrations followed after 13 and 8 months, respectively, followed by a marrow relapse with symptoms of CNS involvement in one case. Both patients died because of treatment resistance (the overall survival time equalled 70 and 44 months, respectively).
We report a case of acute myelomonocytic leukaemia (AMML) who presented with a striking leukaemic infiltration in the frontal area of the scalp that was also rapidly followed by infiltrates in other sites of the skin, mainly in the upper thoracic wall. Leukaemic skin infiltration developed independently of his haematological status; nevertheless, he rapidly progressed to acute leukaemia. We emphasise the importance of early recognition and identification of such lesions with a subsequent early application of systematic antileukaemic therapy.
The skin involvement of the myelodysplastic syndrome (MDS) can take the form of either a neoplastic infiltration or various non specific lesions. The occurrence of these lesions may be the presenting feature of the disease (MDS) or may herald its progression to acute leukemia. Recognition and early diagnosis have therapeutic and prognostic significance.
We present the case of a 62-year-old white female with a history of previously treated angioimmunoblastic T-cell lymphoma, who died suddenly 1 day after presentation for presumed tumor recurrence. Postmortem gross examination revealed a 4-cm cardiac mass that was adherent to the free wall of the right atrium. Histologic examination showed extensive infiltration of the cardiac conduction system by lymphoma, which likely lead to fatal arrhythmia. Histological examination revealed systemic dissemination by an Epstein-Barr virus (EBV)-positive large B-cell lymphoma. Careful examination of the cardiac conduction system on serial sections was crucial in documenting the cause of death due to lymphomatous infiltration. As in this patient, lymphomatous infiltration of the heart may produce sudden cardiac death without obvious premortem signs of cardiac compromise. This case illustrates the utility of examination of the cardiac conduction system in necroscopy studies of sudden death.
This paper presents a short survey of the current status of bone marrow diagnosis in systemic neoplastic disease by means of MR imaging. The different patterns of bone marrow infiltration from lymphomas and leukaemias are presented and the differential diagnoses are discussed. Apart from the primary diagnosis the relevance of conventional MRI and chemical shift imaging for therapy follow up and after-care is discussed.
Interaction of intercellular adhesion molecules (ICAMs) with their receptors has a key role in normal leucocyte adhesion and migration, whereas in leukaemia this has not been well established. In this study, we have evaluated the roles of different adhesion molecules in normal and leukaemia cell extravasation in a novel organotypic model for vessel wall and measured plasma ICAM-1 and -2 levels in acute leukaemia patients at diagnosis and during chemotherapy. We found that both normal mononuclear cells and blast cells from acute leukaemia patients, as well as retinoic acid-treated promyelocytic leukaemia cells, rapidly extravasated through endothelial cell layers into the underlying collagen matrix. ICAM-1 antibody prevented the extravasation, while antibodies to other adhesion molecules showed little (CD18, ICAM-2) or no inhibition (CD11a and ICAM-3). Soluble ICAM-1 (sICAM-1) protein had no effect. We also observed increased plasma sICAM-1 and -2 levels in leukaemia patients and found that they correlated only weakly with the white blood cell count. No correlation was found between sICAM-1 or -2 levels and the response to therapy. Although elevated sICAM-2 levels decreased rapidly during chemotherapy, sICAM-1 levels did not. Because sICAM-1 protein had no effect on leukaemia cell extravasation in vitro, it is probable that the increased plasma sICAM-1 levels in leukaemia patients may not play a role in leukaemia cell infiltration. However, as we showed that ICAM-1 mediated leukaemia cell extravasation on the cell surface, it is possible that cellular ICAM-1 has an important role in disease progression.
A case of B-CLL which was complicated by chronic renal failure due to leukaemic infiltration of the kidney is reported. Treatment with chlorambucil, prednisolone, and renal bed irradiation resulted in a substantial improvement in renal function which persisted until the the patient's death from marrow failure some eight years later. The temporal association between treatments and response suggested that renal bed radiotherapy had contributed to the improvement in renal function. This case is one of only two reported cases in which chronic renal failure due to CLL has been treated with radiotherapy. It is unique in that the renal response was shown histologically. Leukaemic infiltration of the kidney is common in CLL but, characteristically, is not associated with renal impairment. An improvement in renal function has been described in two patients with acute renal failure after chemotherapy.
A 27-year-old pregnant woman was admitted to a local hospital because of headache, nausea, and general fatigue. Her blood examination showed leukocytosis, anemia, and thrombocytopenia. She was referred to our hospital in March 1998. Her bone marrow was normocellular with an excess of blasts (89.1%, peroxidase stain(-), PAS stain(-)) that displayed a positive immunophenotype for CD2, CD4, CD5, CD7, CD34, CD38, and CD71. Chromosome analysis revealed complex abnormal karyotypes. The patient was given a diagnosis of acute lymphoblastic leukemia associated with central nervous system and breast infiltration, and received induction chemotherapy during the second trimester of her pregnancy. After she achieved complete remission, a cesarean section was performed, and a healthy baby delivered. Our experience in this case demonstrated that combination chemotherapy during the second trimester of pregnancy is feasible.
The clinical and haematological analysis is presented of six out of 68 cases of acute lymphoblastic leukaemia in children. In these six cases mediastinal involvement was found. They accounted for 8.8% of all patients and all were boys aged over 4 years. In all these cases the course was dramatic, with high risk and with signs of poor prognosis: infiltration of organs, very high leucocyte counts. T-cell blasts. The evident clinical uniformity of the symptoms and laboratory similarity of these cases suggest that they should be isolated as a separate subgroup, and therapeutic failures observed as yet indicate the necessity of caution in prognosis.
Mucosal lesions are a frequent finding in patients with hematologic malignancies. Early diagnosis and institution of adequate therapeutic modalities can significantly reduce the morbidity and even mortality in these patients. Primary lesions are the consequence of infiltration of the oral tissues by malignant cells. Secondary lesions result from the condition of bone marrow suppression induced by replacement of the blood forming tissue by malignant cells. Lesions caused by the oncologic treatment, directly or indirectly, are called tertiary lesions. This article describes and illustrates the different types of lesions.
A 10-year-old boy was evaluated for fever, weight loss, uveitis, normocytic, normochromic anemia, renal insufficiency, and hypergammaglobulinemia of 8 weeks' duration. Infectious and neoplastic causes of fever were excluded. A renal biopsy performed in the 4th week of disease revealed diffuse plasmocytic interstitial nephritis. No treatment was prescribed and the patient was transferred to another hospital. Because clinical symptoms and renal insufficiency were still present, in the 8th week of disease a second biopsy was performed, which showed lympho-monocytic interstitial nephritis. At the same time, phenotypic analysis of peripheral blood mononuclear cells was carried out, revealing a significantly decreased number of CD3(+), CD4(+), and CD3(+)/CD8(+) cells, increased non-T CD3(-)/CD8(+) and CD56(+) NK cells, and decreased "naïve" (CD45RA(+)/CD4(+)) and memory (CD45RO(+)/CD8(+)) T lymphocytes. A 6-month course of oral prednisone was prescribed. Clinical symptoms and laboratory findings quickly returned to normal values. After 13 days of corticosteroid therapy, a second phenotypic analysis of peripheral blood mononuclear cells was performed, which revealed normalization of CD3(+), CD4(+), and CD3(+)/CD8(+) cells as well as proportions of non-T CD8(+) and CD56(+) NK lymphocytes, "naïve" and memory cells. This case shows spontaneous evolution of tubulointerstitial infiltrates from plasmacytic to lympho-monocytic, profound disturbances of the immunological system, and the beneficial effect of corticosteroids on both the clinical course and immunological disturbances.
A 40 year old man presented with abdominal pain, jaundice, weight loss, and hepatosplenomegaly. Liver function tests revealed cholestatic jaundice and a computed tomography scan showed an enlarged liver, with a normal biliary tree. Liver biopsy showed diffuse infiltration by neutrophils, monocytoid cells, and blasts. Peripheral blood film and bone marrow were consistent with acute myeloid leukaemia. After treatment with chemotherapy using an acute myeloid leukaemia protocol (UK Medical Research Council AML-12), there was complete resolution of jaundice and the patient went into complete molecular remission.