11/279. CD56 blastic transformation of chronic myeloid leukemia involving the skin. We report on two patients with chronic myeloid leukemia (CML) who presented blastic transformation involving the skin, with leukemic infiltrates showing unusual morphologic and immunohistologic characteristics. Both patients were elderly men with a 36-month and a 40-month history of CML, respectively. They presented with disseminated, reddish to violaceous papules and plaques (case 1), and with localized reddish nodules on the left temporal area (case 2). Concurrent features of blastic transformation in the bone marrow were observed in one patient (case 1). Histopathologic examination of skin lesions revealed similar features in both cases. There was a moderate to dense dermal infiltrate composed mainly of medium-sized atypical mononuclear myeloid precursor cells with only few relatively well-differentiated cells of the granulocytic series. Histochemical staining for naphthol-ASD-chloroacetate esterase revealed strong positivity (>50% of neoplastic cells) in case 2 and only scattered positivity (< 10% of neoplastic cells) in case 1. Immunohistologic analysis performed on paraffin-embedded sections showed in both cases variable reactivity of neoplastic cells for leucocyte common antigen (CD45), lysozyme, myeloperoxidase, CD11c, CD15, CD43, CD66, CD68, HLA-DR, and the neural cell adhesion molecule (NCAM) CD56. A negative reaction was observed for CD3, CD34, and TdT. The immunohistologic findings were remarkably similar to those reported for acute myeloid leukemia (AML) with monocytic differentiation (French-American-British [FAB] classification, subtype M4). Examination of blasts from the bone marrow performed in one patient (case 1) revealed a similar phenotype also with CD56 expression. In conclusion, our observations show that specific cutaneous infiltrates in CML may show morphologic and immunohistochemical characteristics similar to those observed in AML with monocytic differentiation. Moreover, specific cutaneous manifestations of CML may express CD56. ( info) |
12/279. Improvement of quality of life after splenectomy in an HTLV-I carrier with T-cell prolymphocytic leukemia. A 34-year-old woman of HTLV-I carrier with T-PLL, whose quality of life improved and survival was prolonged after splenectomy, is described. The patient had marked splenomegaly, generalized lymphadenopathy and marked proliferation of abnormal lymphocytes in the peripheral blood with an irregular nucleus, deeply basophilic cytoplasm and a single prominent nucleolus, which were positive for CD2, CD3, CD5, CD7, CD4 and CD8. Although the patient had serum antibody against HTLV-I, HTLV-I proviral dna integration was not detected. She was diagnosed as an HTLV-I carrier with T-PLL and received combination chemotherapy and 15.1 Gy splenic irradiation. However, the generalized lymphadenopathy and splenomegaly did not improve. The patient underwent splenectomy to palliate abdominal distension and hypersplenism. After the operation, her symptoms improved dramatically and within a week her hemoglobin concentration and platelet count normalized. She was discharged from hospital two weeks after the splenectomy, however 11 months later, she relapsed and despite treatment with chemotherapy and alpha-interferon, she died two months after the second admission. autopsy findings revealed that PLL cells had invaded the bone marrow, lymph nodes, liver, lungs, kidneys, uterus, ovaries and adrenal glands. ( info) |
13/279. Peri-optic nerve infiltration during leukaemic relapse: MRI diagnosis. BACKGROUND: A 10-year-old boy with a history of acute lymphoblastic leukaemia (ALL), but without previous evidence of central nervous system involvement, presented with seizures 3 years after complete remission. MATERIALS AND methods: MRI showed bilateral enlargement of the optic nerves despite normal ophthalmological examination. RESULTS: Only the third cerebrospinal fluid examination showed 2 % blasts without concomitant bone-marrow infiltration. Enlargement of the optic nerves was consistent with bilateral leukaemic peri-optic nerve infiltration. The appearances returned to normal after chemotherapy. CONCLUSION: The optic nerves are a potential site of relapse in patients with systemic and meningeal ALL, even in the absence of ophthalmological signs. ( info) |
| We report a case of chronic myelocytic leukemia (CML) in which the initial manifestation of blastic crisis was massive genital bleeding. The bleeding was diagnosed as being caused by CML metastasis to the uterus on the basis of the magnetic resonance imaging findings. ( info) |
15/279. A new cause of 'non-responsiveness' in coeliac disease? A 42 year old man presented with gluten-responsive coeliac disease and secondary pancreatic insufficiency. Subsequently his symptoms relapsed and repeat small intestinal biopsy showed villous atrophy and infiltration by leukaemic cells, despite continuation of a gluten-free diet. Serious causes of relapse and non-responsiveness in coeliac disease include enteropathy-associated t-cell lymphoma, ulcerative jejunitis and an end-stage hypoplastic mucosa. This is the first report of non-responsiveness due to infiltration by leukaemia. ( info) |
16/279. Acute basophilic leukemia presenting with abnormal liver function tests and the absence of blast cells in the peripheral blood. Acute basophilic leukemia is an uncommon form of acute leukemia, rarely occurring as a de novo disease. We describe a case of de novo acute basophilic leukemia occurring in a 47-year-old man who presented with abnormal liver function tests in the absence of leukemic infiltration of the liver. We postulate that this presentation occurred as a consequence of pathophysiological features of the malignant basophilic blast cells. ( info) |
17/279. Vaginal obliteration after total body irradiation and chemotherapy as treatment for acute myeloid leukemia. Although radiotherapy is an integral part in the management of certain types of hematological malignancies, its effect on the reproductive system has been well documented. We report a rare complication where a patient had complete vaginal obliteration after receiving a dose of total body irradiation (1575 cGy) as part of her treatment for acute myeloid leukemia. A 37-year-old married woman, G3P2, underwent high-dose cyclophosphamide accompanied by high dose (1575 cGy) total body irradiation (TBI) as part of her treatment for acute myeloid leukemia (AML: m1) when she was 35 years of age. After TBI, the patient developed ovarian failure and amenorrhea, which was confirmed by hormonal evaluation. Nevertheless, she did not receive any hormonal replacement therapy and stopped her sexual life for two years. Fortunately, no recurrence of AML was noted. The patient visited our clinic due to difficulty in performing coitus. physical examination showed a 2-cm short and blinded vaginal pouch. She initially received hormonal replacement therapy followed by surgical correction via vaginoplasty and two months of dilatory replacement and frequent coitus with satisfactory result. To our limited knowledge, vaginal obliteration as a complication of condition regimen has never been reported before. In the present case report, it is unclear whether spontaneous vaginal obliteration resulted from chemotherapy, total body irradiation, or another unknown cause such as a concomitant leukemic infiltration of the vaginal wall, severe bacterial and fungal infection before treatment, or from any combination of the above. However, due to this case presentation, we suggest that such patients must receive hormonal replacement therapy and be encouraged to have a normal sexual life to avoid this possible problem. ( info) |
18/279. Clinicopathological features of aggressive large granular lymphocyte leukaemia resemble Fas ligand transgenic mice. Fas ligand triggers cell death after interaction with its receptor Fas. Altered expression of Fas has been associated with lymphoproliferation and autoimmune disorders in both mice and man. apoptosis of lung and liver tissue is seen in Fas ligand transgenic mice. It is not known whether constitutive expression of Fas ligand can cause a similar human disease. Four patients with aggressive large granular lymphocyte (LGL) leukaemia involving lung and liver were studied. All four patients were severely ill with pulmonary involvement. Two patients presented with hypoxia and were oxygen dependent; the other two patients had severe pulmonary hypertension. lung biopsies showed interstitial infiltration by leukaemic LGL. The infiltrating lymphocytes expressed both Fas and Fas ligand, whereas normal pneumocytes expressed only Fas. Similar findings were observed in liver biopsies from these patients. Features mimicking the pathological changes of graft-versus-host disease were observed, including pneumocyte apoptosis. All four patients had high levels of circulating Fas ligand. Successful treatment with oral methotrexate or 2-chlorodeoxyadenosine was associated with disappearance or marked reduction of circulating Fas ligand. These results suggest that dysregulated expression of Fas ligand can lead to human disease with pathological features resembling graft-versus-host disease. ( info) |
19/279. An unusual case of leukemic non-Hodgkin's lymphoma with blastic transformation. We report on a patient who was diagnosed as having B-cell chronic lymphocytic leukemia (CLL) with atypical morphology. flow cytometry disclosed CD5, CD19, and CD23 positivity, an immunophenotype seen mostly in B-CLL. histology of the spleen and bone marrow suggested a diagnosis of small lymphocytic lymphoma. Upon blastic transformation, only 3 years after the diagnosis had been made, unusual clinical and laboratory features emerged. Lymphoid blasts appeared in the peripheral blood, and the patient developed nodular infiltrates consisting of these blasts at recent venous puncture sites. The patient did not respond to chemotherapy and died. The lymphoid blasts in the peripheral blood were CD5-, CD19 , and CD23 and harbored t(11;14) (q13;q32) and t(11;21)(p11;q21) translocations. To account for the possibility of two independent lymphoid malignancies, molecular genetic analyses were performed on samples from the spleen, bone marrow and a lymph node with the large-cell lymphoma, which showed identical clones in these tissues. This unusual case supports the idea that in leukemic non-Hodgkin's lymphoma, in addition to morphology, an accurate diagnostic workup requires immunophenotypic, cytogenetic, and molecular studies. ( info) |
20/279. Secondary cutaneous infiltration in B cell chronic lymphocytic leukemia. We describe a patient presenting with B cell chronic lymphocytic leukemia (B-CLL) who subsequently developed cutaneous infiltrates. Specimens of the blood, bone marrow and cutaneous infiltrations all showed the same heavy-chain gene rearrangement. Following failure of conventional chemotherapy, and in view of the similarity of the disease to cutaneous T cell lymphoma, interferon-alpha therapy was employed with satisfactory results. Introduction of this cytokine to the therapeutic modalities for secondary cutaneous B-CLL would hopefully change the poor outcome of this entity, or at least could produce a better quality of life. Loss of histidine decarboxylase activity in the infiltrating cells - in contrast to circulating lymphocytes - may be associated with the transformation of B-CLL to a more aggressive infiltrative form, offering a possible explanation for tissue invasiveness. The changing character of the disease raises the possibility of a second mutational event in the course of B-CLL. ( info) |