G. J. den Ottolander

Megakaryoblastic Leukaemia (Acute Myelofibrosis): a Report of Three Cases

Three patients with megakaryoblastic leukaemia are described. All three presented with pancytopenia, a few blast cells in the peripheral blood and absence of overt hepatosplenomegaly. In two of them bone marrow aspiration yielded a dry tap. Histological investigation of the bone marrow indicated that the megakaryocytic cell line was the dominant proliferating lineage. Cytochemical and EM investigation supported these findings. The isomorphic isoenzyme pattern of the elevated serum lactic dehydrogenase might be of diagnostic importance. Despite chemotherapy, there was a rapidly fatal terminal leukaemic phase with high blast cell counts. The differentiation from other haematological malignancies, especially acute (aleukaemic) leukaemias and the accelerated phase of primary (chronic) myelofibrosis, is discussed. The picture appears to be identical with acute (malignant) myelofibrosis.

An Identical Translocation between Chromosome 1 and 7 in Three Patients with Myelofibrosis and Myeloid Metaplasia

Summary. An identical chromosome abnormality was observed in three unrelated patients with myelofibrosis and myeloid metaplasia, two of the patients showing a history of polycythaemia vera (PV) before development of the myelofibrosis. Unstimulated peripheral blood cultures showed a translocation between chromosomes 1 and 7 replacing a homologue of pair 7. It was identified by G‐ and C‐banding as t(1;7)(7pter→7p11::1p1?→1qter).

The HLA-system in immune thrombocytopenic purpura: its relation to the outcome of therapy

Summary. The distribution of HLA‐A, ‐B, ‐C and ‐DR antigens was investigated in 154 patients with immune thrombocytopenia (99 adults and 55 children). An increased frequency of HLA‐Bw56 (a split of HLA‐Bw22) was found both in the adults (RR = 4.30) as well as in the children (RR =7.91). Differences in HLA antigen distributions have also been analysed in patient subgroups with a good and a bad response to corticosteroids and splenectomy. The frequency of HLA‐DR4 was low in those patients with a good response to prednisone as compared with those with a poor response. The opposite was found in splenectomized patients. In 22 patients who did not respond to splenectomy, LB4 (a local split of HLA‐DR4) was completely absent. These results suggest that HLA‐DR4 (LB4) may be a predictive marker for therapy in ITP patients, i.e. a bad response to corticosteroids and a favourable outcome of splenectomy. However, none of the differences were significant after correction was made for the number of antigens tested.

Reactive hemophagocytic syndrome as a presenting feature of Hodgkin's disease

Abstract We report the case of a 60-year-old man with febris of unknown origin, severe pancytopenia, and rapidly developing splenomegaly due to reactive hemophagocytic syndrome and Hodgkins disease. Reactive hemophagocytic syndrome is often rapidly fatal and, once this diagnosis is considered, an underlying infection or malignancy should be treated promptly. An extensive search of the literature revealed only two other cases of reactive hemophagocytic syndrome and Hodgkins disease. This is the only reported patient who survived after being diagnosed as having reactive hemophagocytic syndrome and Hodgkins disease.

Clinical differential diagnosis of hairy-cell leukaemia.

The data on hairy-cell leukaemia (HCL) and resembling disorders in the literature and in our patients were analyzed to determine which clinical features and laboratory data are important for the recognition of HCL in an early stage. In pancytopenic patients the typical pattern of bone marrow involvement in HCL and the low number of monocytes in the peripheral blood appear to be essential for the differential diagnosis. In patients with many neoplastic cells in the peripheral blood, the presence of neutropenia and monocytopenia as well as tartrate-resistant acid phosphatase activity in the neoplastic cells, appears to be crucial for early diagnosis. Thus, the clinical features and routine laboratory data alone are sufficient in the majority of cases to suggest the diagnosis HCL. The monocytopenia proved to be most helpful in this respect. Nevertheless, in all patients, and certainly in patients presenting with atypical features, a bone marrow biopsy is indispensable for the correct diagnosis.

On the presence of reverse transcriptase in myelo- and lymphoproliferative disorders

Buffy coats from 31 patients with a diagnosis of leukemia and 16 normal donors were tested for the presence of a viral‐like reverse transcriptase. Eighty‐five percent of fresh leukemic buffy coats were positive. Also tested were spleens from 16 patients with hematological disorders and 5 spleens from patients without history of hematological malignancy. The 5 normal spleens were negative. Also negative were 4 spleens from patients with Hairy cell leukemia. From the remaining 12 spleens 7 were positive. Reverse transcriptase measurements can be used to distinguish leukemic from normal buffy coats.

The hemalog D automated differential counter in the diagnosis of hairy cell leukemia

We studied the peripheral blood of 37 patients with hairy cell leukemia (HCL) prior to (n = 24) or following (n = 19) splenectomy, in the Hemalog D multi-channel white cell differential counter, to investigate whether the apparatus could contribute to the (early) diagnosis of this entity and to the differential diagnosis of HCL from atypical hairy cell leukemia (AHCL; n = 9), chronic lymphocytic leukemia (CLL; n = 21) and leukemic non-Hodgkin lymphoma of low-grade malignancy (LNHL; n = 19). HCL showed almost invariably monocytopenia, neutropenia and an increased percentage of LUC, with a rather typical picture of the X-Y display of the peroxidase channel. The percentage of hairy cells closely correlated with the percentage of the LUC from the Hemalog D. Discriminant analysis using several parameters of the Hemalog D differential count resulted in a complete separation of HCL from CLL, and a fair, although not complete, distinction of HCL from AHCL and LNHL. It was impossible to discriminate between AHCL and LNHL. The most important discriminating (single or combined) parameters were the absolute monocyte count, the TWBC and the absolute neutrophil number. It is concluded that the Hemalog D is a valuable tool in the (early) diagnosis of HCL and in the discrimination between HCL and other leukemic lymphoproliferative disorders.

Electron microscopical and biochemical investigations on retra viruses in spleen tissue in malignancy

SummarySimultaneous biochemical and electron microscopical investigations on surgically removed spleens yielded evidence for the presence of reverse transcriptase containing (Retra) virus in two patients with hematological malignancies with spleen involvement. In three other patients with hematological diseases and in one control patient, the spleens were negative in both assays. The results of these combined studies support the view, that retraviruses are present in human malignancies.

Dendritic reticulum cell sarcoma: a rare tumor of the follicular compartment.

Recent developments in immunology have revealed that non-Hodgkin lymphomas may be considered as neoplastic counterparts of the reactions that normally take place after antigenic challenge in special compartments of the lymph nodes. For instance, the relationship between the follicular compartment and follicle centre cell tumors has been demonstrated clearly in the literature (Lukes and Collins, 1973; Lennert, 1973; Lennert, 1978a). Three types of cells are present in the follicular compartment: lymphoid cells, macrophages and dendritic reticulum cells (DRC). Thus theoretically three types of “lymphomas” arising from the follicular compartment are possible, i.e. follicle centre cell lymphomas originating from the lymphoid cells, histiocytic sarcoma or true histiocytic lymphomas arising from the histiocytic reticulum cell, and dendritic reticulum cell sarcoma, a tumor in which the neoplastic cell is a DRC (Lennert, 1978b). In a tumor of one of the cell types the other 2 types of cells can also be found among the tumor cells.

A 43-year-old woman with severe pancytopenia and a splenic hematoma

In July 1993, a 43-year-old woman was hospitalized because of a large hematoma of the spleen and pancytopenia. This Caucasian woman, a resident of the Republic of South Africa, was vacationing in her mother country, The Netherlands. In 1991 blood studies had revealed severe pancytopenia. A subsequent bone marrow biopsy showed extensive hypoptasia of the marrow without infiltration of abnormal cells. A diagnosis of aplastic anemia was established. Since then the patient has suffered many infections related to neutropenia (values below 0.5 x 109/1), including relapsing cellulitis with abscess formation on the legs, which required surgical interventions, and candidiasis of the left eye, which resulted in almost total blindness. Because of these severe infections she had received several courses of GM-CSF (Leucomax) which led to a temporary increase in the neutrophil count. However, androgen therapy did not produce any improvement in the pancytopenia. For prevention of infections she was on maintenance therapy with ciprofioxacin. At the time of admission she required blood transfusions every 3-4 weeks. Upon admission, the patient complained of progressive fatigue, shortness of breath, and malaise. During the course of the night before admission severe pain had developed behind the left costal margin. The pain increased with inspiration and coughing. Three weeks before, she had suffered an episode of diarrhea with collapse. She denied any traumata. In the preceding few months she had experienced some discomfort in the left upper quadrant of her abdomen. The remaining history revealed no abnormalities. There was no history of drug or alcohol abuse. She had not noticed any loss of weight. At examination the patient looked very pale and was obviously suffering pain. She had a regular pulse of 88 beats per minute and a blood pressure of 110/90 mmHg, temperature 37.4 ~ C. Respiration was superficial. There were no signs of lymphadenopathy. The liver was not enlarged. The left upper quadrant of the abdomen was extremely tender. Auscultation revealed normal peristalsis. Her legs were covered with subcutaneous hematomas and scars.