Mononucleosis syndrome and acute monocytic leukemia

The association of infectious mononucleosis and an immunocompromised host such as occurs in acute leukemia is reported. The most common cause of infectious mononucleosis is Epstein-Barr virus (EBV) and cytomegalovirus (CMV). Patients with mononucleosis syndrome caused by other agents are rare. We report a case of acute monocytic leukemia (AMoL) who developed varicella zoster virus (VZV) mononucleosis syndrome in the bone marrow recovery phase after myelosuppression due to high-dose cytarabine. Mononuclear leukocytes appearing during the mononucleosis syndrome were very similar to the initial leukemic cells. Varicella zoster virus mononucleosis syndrome was confirmed by delayed herpes zoster rash with dermatomal distribution.     

The distinctive features of acute monocytic leukemia

Acute monocytic leukemia is an uncommon form of acute leukemia. Distinctive clinical features include gingival hypertrophy, lymphoadenopathy, coagulation disorders, and lysozymuria. Blast cell morphology and cytochemistry are diagnostic. Receptors for the Fc fragment of IgG have been demonstrated on the basis of a few cases. The drug VP 16–213 has been shown to be very effective in treatment of untreated and previously treated patients.     

Acute monocytic leukemia complicating combined-modality therapy for localized childhood Ewing's sarcoma

Summary Rapidly fatal acute monocytic leukemia occurred in an 11-year-old boy 33 months after the beginning of irradiation and chemotherapy for nonmetastatic pelvic Ewing's sarcoma. At autopsy, no recurrent primary disease was seen. An analysis of this case together with a review of the literature suggests therapy-related leukemogenesis. Thus, the decline in mortality rate for childhood cancer may be accompanied by an increased incidence of second neoplasms in cured children having the potential of a normal life span.     

Chronic monocytic leukemia terminating in blastic transformation

Summary Chronic monocytic leukemia (CMoL) is a rare disorder, closely related to malignant histiocytosis, but a separate entity. The clinical course of the patient described in this case report was characterized by persistent monocytosis without response to cytotoxic therapy, and a large number of infections. On two occasions a clone of cells containing an extra chromosome 20 was observed. The disease terminated in a phase with rapidly increasing numbers of immature monocytoid cells, corresponding to blastic transformation.     

Two murine monoclonal antibodies to peripheral blood monocyte differentiation antigens discriminate within M5 acute non-lymphoid leukemia (ANLL) cells

Two murine monoclonal antibodies (MoAbs), LAM3 and LAM7 of the IgG₁ isotype, which were produced by immunization with normal peripheral blood monocytes (PBM), were assayed in their specificity by indirect immunofluorescence against a panel of normal as well as leukemic cells. Both LAM3 and LAM7 were reactive with PBM while LAM3 also recognized platelets. Neither MoAb showed reactivity with erythrocytes, granulocytes, or resting and mitogen activated B and T lymphocytes. The reactivity with bone marrow cells correlated with the degree of monocyte contamination. Among the 62 cases of leukemia tested, which included three cases of B-CLL, 19 cases of ALL, and 40 cases of ANLL, both MoAbs reacted highly homogenously only with M5b ANLL cells. These findings indicate that the two MoAbs, which recognize two distinct epitopes, represent useful markers in the differential diagnosis of M5b ANLL.     

Identification of monocytic nature in acute undifferentiated leukemia by in vitro marrow culture study

Summary We report a case with acute undifferentiated leukemia whose leukemic blasts lacked morphological, cytochemical and immunological features of lymphoid or myeloid differentiation. The in vitro culture study defined her leukemia as of monocytic origin. Her marrow blasts underwent monocytic differentiation with strong nonspecific esterase activity when cultured in a liquid system with human placental conditioned medium. The semisolid agar culture showed an AML-type growth pattern. The present study indicates that in vitro culture study can be used as a supplement to improve the classification of certain unclassifiable leukemias.     

Spontaneous remission of acute monocytic leukemia after infection with Clostridium septicum

Spontaneous remissions of acute myeloid leukemia (AML) have been reported in association with infection. Here, we report a case of spontaneous remission of AML in a 47-year-old Saudi Arabian male patient who presented with a few weeks history of recurrent abdominal pain, vomiting and fever. He was diagnosed with acute monocytic leukemia (AML, FAB M5b) and a perforated bowel. He also had Clostridium septicum bacteremia and thus chemotherapy was deferred. He received supportive therapy and intravenous antibiotics. Six weeks later, he achieved spontaneous and complete remission lasting for about 4 months. The remission and relapse were documented by bone marrow examination. Similarly, previous reports of spontaneous remission of AML were short lived and were followed by relapse and progression.     

异基因外周血造血干细胞移植治疗急性单核细胞白血病的临床观察

目的评价异基因外周血造血干细胞移植（allo-PBSCT）治疗急性单核细胞白血病（M5）的疗效,并探讨其并发症的预防及处理。方法 16例M5患者接受allo-PBSCT,其中亲缘11例,非亲缘5例。预处理方案：9例采用清髓方案BUCY,7例采用非清髓方案FBC。亲缘的11例均采用环孢素＋短程甲氨蝶呤预防移植物抗宿主病（GVHD）,非亲缘的5例均采用环孢素＋甲氨蝶呤＋吗替麦考酚酯＋ATG。输注的外周血干细胞有核细胞中位数为6.58×108/kg,CD34＋细胞中位数为4.46×106/kg。结果 16例患者中15例均证实植活,余1例在移植早期因HVOD死亡。植入病人中白细胞植入中位时间为13（9~17）d,血小板〉20×109/L的中位时间为16（8~26）d。发生急性GVHD 6例（Ⅰ度4例,Ⅱ度2例）,发生局限性慢性GVHD 7例。目前无病存活10例,中位生存期为45（3~78）个月。结论 Allo-PBSCT是治疗M5的有效手段,并发症少,能有效延长患者生存时间。     

Acute lymphocytic leukemia: Correlation of clinical features with immunocytochemical classification

Many immunologic studies of acute lymphocytic leukemia (ALL) during the past decade have demonstrated the close correlation of immunologic phenotypes of ALL subclasses with the clinical presenting features and prognosis. However, the clinical application of conventional immunologic techniques had been very limited because of the requirement of a fresh sample to prepare the mononuclear cell suspensions for study. We studied 81 cases of ALL using immunoperoxidase stain for nuclear terminal deoxynucleotidyl transferase (TdT) and immunoalkaline phosphatase stain for surface markers (using monoclonal antibody J₅ for common ALL antigen [CALLA], Leu-1 for pan-T antigen, and B₁ for pan-B antigen) on air-dried smears. The cases were classified as common ALL (TdT⁺, CALLA⁺, pan-T^?, and pan-B^?) (41 cases), null-ALL (TdT⁺, CALLA^?, pan-T^?, and pan-B^?) (19 cases), T-ALL (TdT⁺, CALLA^?, pan-T⁺, and pan-B^?) (nine cases), B-ALL (TdT^?, CALLA^?, pan-T^?, and pan-B⁺) (six cases), pre-B-ALL (TdT®, CALLA⁺, pan-T^?, and pan-B⁺) (four cases), or pre-T-ALL (TdT⁺, CALLA⁺, pan-T⁺, and pan-B^?) (two cases). This subtyping of ALL correlated well with known clinical presenting features, prognosis, chromosome analysis in 35 cases with an abnormal clone, and conventional immunologic typing in 38 cases. The data suggest that these simple and practical immunocytochemical stains can be used for immunologic subclassification of ALL.     

Three cases of familial hairy cell leukemia

In a 10-year interval, a total of 12 cases of familial hairy cell leukemia have been published. They were noted in first degree relatives, mostly in men. In some instances, when the HLA type was performed, a specific HLA type was found in the studied family, but a different haplotype was seen in other families. It appeared that familial cases of hairy cell leukemia were not associated with a “specific HLA antigen” and other factor(s) such as environmental, or some kind of occupational exposure, were suggested to play a role in the familial occurrence of hairy cell leukemia. We add three more familial hairy cell leukemia cases which are different from other published cases, showing a female predominance. The HLA typing revealed interesting findings. The HLA type shared by case 1 and 3 was A2, A30/31(19), B27, Bw4, Bw6. From these, HLA A2, Bw4, and Bw6 were previously reported (Ward FT, Baker J, Krishnan J, Dow N, Kjobech CH: Cancer 65:319–321, 1990). Case 2, shared with the other two the antigen Bw6. Its specific HLA type was A3 and B7, the type previously reported in a family (Begley CG, Tait B, Crapper RM, Briggs RG, Brodie GN, Mackay IR: Leuk Res 11:1027–1028, 1987). Based on these observations, we may conclude that a “specific HLA type,” A2, Bw4, Bw6 and A3, B7 might have a role in the genetic predisposition for hairy cell leukemia. © 1993 Wiley-Liss, Inc.     

Clinical importance of bone marrow monocytic nodules in patients with myelodysplasia: retrospective analysis of 21 cases

Bone marrow monocytic nodules (MNs) can occur in various myeloid disorders. This retrospective review identified 21 patients with myelodysplasia who had unusual and distinct MNs. Eight patients had chronic myelomonocytic leukemia (CMML); 4 had acute myeloid leukemia (AML); and 9 had myelodysplastic/myeloproliferative diseases. In each case, the cells forming MNs expressed strong CD68. MNs appeared to persist even after aggressive chemotherapy, including conventional chemotherapy for 2 AML patients and high-dose chemotherapy preceding allogeneic bone marrow transplantation for 1 CMML patient. Thirteen of 21 patients (62%) died, and acute leukemic transformation was the main cause of death in 3 of 8 patients with CMML. The median survival of the 20 patients with appropriate follow-up was 9.8 months. Our findings demonstrate that MNs are associated with CMML, AML, myelodysplastic syndromes, and myeloproliferative diseases and suggest that MNs are resistant to intensive chemotherapy and patients with bone marrow MNs have a poor prognosis.     

Acute lymphoblastic leukemia: A study of 25 cases by scanning electron microscopy

Summary Cells from 25 cases of acute lymphoblastic leukemia (ALL) were studied under the scanning electron microscope (SEM). In 24 of the cases, the vast majority of circulating leukaemic cells had few microvilli. Villous cells were rarely encountered and prominent ridge-like profiles and ruffled membranes were not seen. Only six cases were studied by immunological techniques and four of the cases were of the null type while in two the cells bore detectable T-markers. It seems that ALL is almost always associated with the presence of cells with few microvilli in the peripheral circulation, differing in this respect from most cases of CLL. Although circulating leukaemic lymphocytes with few microvilli are sometimes seen in CLL, the most frequent cell type encountered is a more villous lymphocyte.Differences between leukaemic cells from patients with ALL, CLL and non-lymphoblastic leukaemias are discussed. It appears that SEM may help to distinguish lymphoblastic and nonlymphoblastic leukaemic cells in many instances and can be used as a useful adjunct to other modes of microscopy in the diagnosis of acute leukaemia.

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Zusammenfassung Bei 25 Patienten mit akuter Lymphoblasten-Leukämie (ALL) wurden die Leukämiezellen mit Hilfe des Raster-Elektronenmikroskops untersucht. Bei 24 Patienten fanden sich überwiegend glatte Zellen mit nur wenigen Mikrovilli. Bei diesen Patienten wurden nur wenige zottige Zellen gefunden; Zellen mit prominenten Streifenprofilen und gefalteten Membranen wurden nicht beobachtet. 6 Fälle wurden zusätzlich mit immunologischen Techniken untersucht; in 2 Fällen waren T-Zell-Marker nachzuweisen, 4 gehörten zur sogenannten Null-Fraktion. Die ALL zeigt also fast immer glatte Zellen, im Gegensatz zu den meisten Fällen von chronisch-lymphatischer Leukämie (CLL). Bei der letzteren sind zwar gelegentlich auch glatte Zellen zu sehen; häufigster Zelltyp ist jedoch eine stärker mit Zotten versehene Zelle.Die Unterschiede der Zelloberfläche bei CLL, ALL und anderen Leukämien werden besprochen. Die Raster-Elektronenmikroskopie erlaubt in vielen Fällen eine Unterscheidung zwischen lymphatischen und nicht-lymphatischen Zellen und ist eine wertvolle Ergätnzung zu anderen mikroskopischen Methoden bei der Leukämie-Diagnose.

     

Acute basophilic leukemia: case report

The term "basophilic leukemia" has been in use for 75 years. However, consistent diagnostic criteria are lacking. This is due to the rarity of the disease and to the routine unavailability of special tests that are often required to confirm a diagnosis. We report an unusual case of acute basophilic leukemia in a child who was referred to our Center, arriving with partially treated acute lymphoblastic leukemia. Basophilic differentiation on light microscopy was evident from the coarse basophilic granules in blasts, a progressive maturation of blasts toward basophils, and toluidine positivity on cytochemistry. Blasts showed a myeloid immunophenotype (CD13+, CD33+, CD117+) with a characteristic dual positivity for CD34 and CD25, highly suggestive of basophilic nature of the blasts. Conventional cytogenetic studies revealed translocation t(8;21)(q22;q22). A diagnosis of acute basophilic leukemia with t(8;21) was made. Review of pre-therapy slides showed features consistent with AML-M2 with basophilia. There were no basophilic blasts. With these features, a diagnosis of acute basophilic leukemia secondary to AML-M2 was made. In our patient, basophilic leukemia appears to have evolved from selective clonal proliferation of "basophil-committed blasts" during the course of the disease in a case of AML-M2 with basophilia.     

Acute nonlymphocytic leukemia with eosinophilic differentiation

Four cases of de novo acute nonlymphocytic leukemia (ANLL) with early eosinophilic differentiation are described. The clinical course did not differ from that of the usual forms of ANLL. Morphologic and cytochemical features that can support this diagnosis are discussed. Particularly, the cyanide-resistant peroxidase stain appeared to be a specific marker of eosinophilic differentiation. Acute eosinophilic leukemia is a distinct entity, and this unusual subtype of ANLL can be set apart from other forms of ANLL characterized by hypereosinophilia.     

Tetrahydrofolate dehydrogenase cytochemistry in acute lymphoblastic leukemia

We studied the cytochemical distribution of tetrahydrofolate dehydrogenase (FH4D), an enzyme involved in nucleic acid metabolism and thus in cell proliferation and differentiation processes, in bone marrow blasts from 37 cases of acute lymphoblastic leukemia (ALL), of whom 23 were pediatric patients. 26 cases were analyzed at onset, 11 in relapse. The ALL cases were immunologically classified as T (10), common (20), B (3) and null (4). In each subgroup the majority of lymphoblasts were positive, with heterogeneous positivity patterns and variable degrees of enzyme activity. Most T lymphoblasts were characterized by focal localization of FH4D, whereas in common blasts reactivity - usually less strong - was either focally localized or scattered with several fine granules. Finally, many B and null blasts showed diffuse positivity. A quantitative evaluation of FH4D activity using cytophotometric technique (Vickers M86) demonstrated higher degrees of reactivity in leukemic blasts than in normal lymphocytes. Moreover, slightly different levels of reactivity were observed in relation to immunological phenotype, age and stage of the disease. Therefore we think that FH4D is a useful additional marker for ALL characterization.     

Acute basophilic leukemia

Abstract: Acute basophilic leukemia has recently been included into a revised classification of acute leukemias proposed by the WHO panel. Due to the rarity of the disease, consistent diagnostic criteria are lacking. We report on two cases of acute basophilic leukemia that occurred in our department during the last 10 yr. We focus on their clinical, morphological and cytogenetic presentation. Both patients were >60 yr of age, and presented in good clinical condition with alterations to their full blood count. None had cutaneous symptoms such as erythema or urticaria. Cytogenetic analyses in the first patient showed a normal karyotype, while the second displayed a translocation t(2;6); (q23?4;p22?3), as well as a del (12)(p11). Earlier observations have linked bone marrow basophilia either to a deletion of the short arm of chromosome 12 (p11–13), to translocations involving the long arm of chomosome 6 at 6q23 or to the translocation t(6,9); (p23;q34). However, other translocations involving chromosome 6p23 have not been described before. Treatment of our patients consisted of supportive treatment in the one with normal karyotype and aggressive chemotherapy in the other patient. Both patients died within one year after diagnosis due to progressive or recurrent leukemia.     

Heavy-chain immunoglobulin gene rearrangement and cytoplasmic immunoglobulin expression in acute monocytic leukemia following primary germ cell tumor

A case of acute monocytic leukemia with rearrangement of the immunoglobulin heavy-chain gene and strong cytoplasmic immunoglobulin expression in a young patient treated with multi-drug chemotherapy for primary seminomatous germ cell tumor 13 months earlier is reported. The short latency period from the beginning of therapy for primary germ cell tumor and the abrupt onset of leukemia with no identifiable prodrome bear similarities to podophyllotoxin-related leukemias.Supported by a grant from theFonds zur Förderung der wissen-schaftlichen Forschung (P09044-MED)     

急性单核细胞白血病M5a和M5b核型与临床特征的比较

目的比较急性单核细胞白血病M5a和M5b核型差异,并了解其与临床特征之间的相互关系.方法采用骨髓直接法和24 h短期培养法制备染色体标本,用G显带技术,对58例成人初发急性单核细胞白血病进行核型分析,同时对其临床资料进行回顾性研究.结果58例患者中正常核型28例,异常核型30例,其中,正常核型在M5b中出现率高于M5a(P＜0.01),异常核型中11q23异常和+8染色体在M5a中均较M5b常见(P＜0.01);临床上异常核型的M5患者常有高白细胞计数,中枢神经系统浸润,完全缓解率低及存活期明显缩短的特征.结论急性单核细胞白血病在遗传和临床上是一组异质性疾病,但M5a和M5b仍具有各自独特的遗传学背景.