Biomedical subjects
Publications and source records attributed to R K Woodruff.
The case of a 69-year-old man with IgGK myeloma in whom meningeal myelomatosis with multiple cranial nerve palsies developed, is reported. Review of previously reported cases of clinically apparent meningeal myelomatosis indicates this rare complication frequently presents with cranial nerve palsies; currently available treatment is ineffective.
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The criteria proposed by the French-American-British (FAB) Group for the diagnosis of acute erythroleukaemia (AEL), including the requirement for greater than or equal 30% marrow myeloblasts, were used in a review of patients with erythroleukaemia. Ten patients with AEL were identified, and a further twelve patients with marrows suggestive of AEL but having less than 30% myeloblasts were classified as having refractory anaemia with excess of blasts (RAEB). The AEL patients had a poor prognosis, poor response to chemotherapy, and none showed evolution to myeloblastic or monoblastic leukaemia. In contrast, the patients with RAEB appeared to survive longer, respond better to chemotherapy, and several evolved into typical myelogenous leukaemia. The FAB criteria subdivides patients with erythroleukaemia into groups with apparent clinicopathological and prognostic differences. Further studies using these or equally strict criteria are required.
A fourteen-year-old boy presenting with marked acanthocytosis of the red blood cells and a compensated haemolytic anaemia was shown to have the McLeod blood type. Only three other individuals have been reported as having this abnormality of the Kell blood group system without evidence of chronic granulomatous disease. Serological typing for the McLeod phenotype should be undertaken in males with unexplained acanthocytosis, and CPK estimation may provide a screening test for this condition.
The results of detailed studies of 3 patients with essential thrombocythaemia (ET) are presented. Qualitative tests of platelet function were abnormal, and autologous platelet survivals were diminished. Megakaryocyte ploidy distribution analysis showed an increased proporttion of cells with high ploidy, and buoyant density distribution analysis showed increased proportions of less dense platelets. Ultrastructural studies of the platelets showed proliferation of the dense tubular system with deficiency of the surface-connected canalicular system. Megakaryocyte electron microscopy showed abnormal distribution of demarcation membrane complex and granules. The precise functional abnormality of the platelets in ET has not yet been defined, but a release defect of variable severity with corresponding morphological abnormalities of dense tubular and surface-connected canalicular systems, seems the most likely explanation.
Forty-four patients with Ph positive leukemia (36 developing blast crisis after chronic phase and eight presenting in acute leukemia) were classified into subgroups on the basis of reactivity of blasts with an anti-serum made against non-T,non-B acute lymphoid leukemia (ALL+), levels of terminal transferase enzyme (TdT+) and morphology. Positivity with anti-ALL serum was the most sensitive and reliable marker, and TdT was an important aid. The presence of "lymphoid" blasts in blast crisis of CML was related to the response to chemotherapy incorporating Vincristine and Prednisolone (VP). Patients with ALL+ blasts frequently (14 of 15 cases) responded to therapy while 21 of 25 patients who had no ALL+ blasts failed to respond. The clinical course of the ALL+ patients was variable: eight patients remitted with return to the appearances of the chronic phase; four patients demonstrated elimination of the Ph1 positive clone with hypoplasia and this was followed by normal (Ph1 negative) marrow regeneration in two. Subsequent relapse was of either the ALL+ "lymphoid" or the ALL-myeloid type. A regimen incorporating VP should be the treatment of choice in "lymphoid" blast crisis of CML.
The details of 16 patients with extramedullary plasmacytoma are presented. All but one presented with localized disease, and all were treated successfully by radiotherapy. Only one patient developed local recurrence and only one has developed disseminated disease. Radiotherapy is recommended as the primary treatment of choice for these localized radiosensitive tumors. It is suggested that the good prognosis of plasmacytomas of the head and neck, in contrast to plasmacytomas arising at other sites, is related to the observation that a high proportion of these tumors are localized at the time of diagnosis and that very large primary tumors are uncommon.
The clinical features and the results of treatment of twelve patients with solitary plasmacytoma of bone (SPB) are reported. The median age at presentation was 53 years and there was a predominance of males. Five patients have developed multiple myeloma (MM), for within three years and one after twelve years. Seven patients have not shown evidence of dissemination, and five are alive 1--15 years (median 9 years) after diagnosis. Two patients had metastases to local lymph nodes. There are no reliable criteria by which patients with truly SPB can be prospectively distinguished from those with occult MM, and it is recommended that all patients with apparent SPB should receive "curative" local therapy.
Analysis of 237 patients with myeloma, retrospectively classified according to the staging system proposed by Durie and Salmon, showed highly significant differences (P less than 0.0001) in the survival of patients with low, intermediate and high tumour cell masses (Stages I, II and III respectively). The median survival for Stage I patients was 64 months, Stage II 32 months, and Stage III 6 months. The median survival of patients presenting with renal impairment was only 2 months, compared to 21 months for those presenting with normal renal function (P less than 0.0001). The use of this clinical staging system, which is based on myeloma protein synthesis rates and the calculation of total body tumour cell mass, should be considered in planning future trials of therapy in this disease.
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Fifty-one adults with acute lymphoblastic leukaemia were entered into a trial of intense initial chemotherapy and early "prophylaxis" of the central nervous system (CNS). Initial treatment with OPAL (Oncovin (vincristine), prednisolone, adriamycin (doxorubicin), and L-asparaginase (colaspase)) followed by craniospinal or cranial irradiation and intrathecal methotrexate produced remission in 36 patients (71%). Seventeen of these patients relapsed three to 18 months after the start of remission; the remainder had been in remission for 12 to 52 months by the end of the study. The predicted median duration of complete remission was 18.5 months. None of the four patients who initially had clinical evidence of CNS disease, three of whom also had leukaemic cells identical to those found in Burkitt's lymphoma, achieved remission. Those patients who initially had hepatomegaly or splenomegaly had a shorter remission than those without. The predicted median survival was 27 months in those who achieved complete remission, one month in those who did not, and 21 months overall. The addition of colaspase and doxorubicin to vincristine and prednisolone and the use of early CNS treatment clearly improved the remission rate among adults with acute lymphoblastic leukaemia, though the presence and length of remission was affected by the extent of disease at presentation. Burkitt-like leukaemia, which had a poor prognosis, is probably a separate disease and may benefit from a different therapeutic approach.
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Fifteen patients presenting with plasma cell leukemia (PCL) are reported in detail. The clinicopathologic features of PCL differ from typical myeloma and resemble those of acute leukemia: patients with PCL have less bone disease but a much higher incidence of organomegaly and tissue infiltration as well as diffuse marrow involvement and more pronounced pancytopenia. One of the reported patients developed meningeal plasma cell leukemia and is reported in detail. Cytomorphologic assessment of PCL cells showed nuclear immaturity and obvious nuclear/cytoplasmic asynchrony. Despite the use of cytotoxic agents known to be effective in myeloma, the prognosis in PCL is poor, and the median survival of the reported patients was only 2 mo.
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Three patients with acute lymphoblastic leukaemia are reported in whom Hodgkin's disease developed during chemotherapy-maintained remission. This association has been reported only once before. The pathogenesis of the Hodgkin's disease in these patients is discussed, including the possible role of anti-leukaemic therapy.
Terminal deoxynucleotidyl transferase (T.D.T.) has been measured in the cells of 112 leukaemia patients whose cells were characterised by membrane markers as well as by standard haematological and cytochemical criteria. The concentration of enzyme ranged from 0.1 to 1.4 units/10(8) cells in the bone-marrows of 20 patients with normal marrow or benign marrow hyperplasia. The enzyme level was raised in 30 of 31 patients with untreated acute lymphoblastic leukaemia (A.L.L.) whose cells reacted positively with an anti-A.L.L. serum (range 0.9-197, mean 59, units/10(8) cells) and in all 9 patients with thymic A.L.L. (Thy-A.L.L.) (range 1.5-95, mean 36, units/10(8) cells). All but 1 of 17 patients with acute myeloblastic leukaemia gave negative results (range 0-1.5, mean 0.60, units/10(8) cells), and T.D.T. was also normal in all 7 bone-marrow samples from patients with chronic granulocytic leukaemia (C.G.L.) in the chronic phase. The T.D.T. assay gave a clear distinction between 11 patients with C.G.L. in lymphoid transformation, anti-A.L.L.-serum positive (range 15-226, mean 83, units/10(8) cells), and 12 patients with C.G.L. in myeloblastic transformation, anti-A.L.L.-serum negative (range 0-1.9, mean 0.7, units/10(8) cells). Among 17 patients with otherwise unclassifiable acute leukaemia, 10 gave raised values (range 1.6-113 units/10(8) cells) and 7 gave normal values. It is concluded that the assay of T.D.T. in the peripheral blood or bone-marrow of patients with acute leukaemia is of value in differentiating lymphoid (including non-T non-B and Thy-A.L.L.) from myeloid leukaemia.