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After briefly recalling the cytomorphological aspects of Pelger-Huet anomaly, its statistical occurrence, the modalities of hereditary transmission and its differentiation from pseudo-Pelger, a case of the homozygotic variant is reported. The practical and theoretical importance of the anomaly is commented on briefly.
A homozygot carrier of a congenital nuclear anomaly of blood cells/Pelger-Huet's anomaly: abnormal segmentation of granulocytic leukocytes) is described. The morphologic analysis of blood samples in 4 family members showed similar Pelger-Huet's anomalies. The phagocytic activity, the "killing test" and the inhibition test of leucocyte migration of the patients neutrophils were in normal ranges.
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Pelger-Huet's granulocyte abnormality is discussed. This abnormality is characterized by the presence of granuloyctes with a non-segmented nucleus and thick nuclear chromatin gathered in large lumps, in contrast with the "youthful" appearance of the nuclei. In humans, a real Pelger-Huet abnormality with familial character and dominant autosomic transmission is distinguished together with a pseudo-Pelger in which the granulocytes assume cytological features similar to those typical of the constitutional abnormality but whose genesis is closely correlated to the disease of which these are an epiphenomenon.
A survey of the relevant literature is followed by the presentation of a case of transient, acquired Pelger-Huët anomaly observed in a patient operated for Crohn's disease.
The syndrome of Shwachman is characterized by pancreatic insufficiency and bone marrow dysfunction, usually manifesting itself as neutropenia. The pancreas shows replacement of the exocrine glands by adipose tissue; sweat electrolytes are normal. A 23-year-old male who was known to suffer from neutropenia and pancreatic dysfunction from early childhood, presented with fever, acquired Pelger-Huet anomaly (of the polymorphonuclear granulocytes) and sideroblastic anaemia, a combination of symptoms suggestive of preleukaemia. A few months later he died of acute myeloblastic leukaemia and autopsy showed a dystrophic pancreas. Considering this case history it seems possible that the haematological anomalies of Shwachman's syndrome are signs of preleukaemia. Careful follow-up of patients suffering from Shwachman's syndrome seems warranted.
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Decreased nuclear segmentation and coarse chromatin only in mature eosinophils was seen in a patient with acute myeloblastic leukemia. These eosinophils were considered to have acquired Pelger-Huët anomaly. On cytochemical study they displayed a strong PAS-positive reaction.
Hyposegmentation of granulocyte nuclei was diagnosed in an adult dog after unsuccessful attempt at finding an occult chronic infection. Intermittent signs of prostatitis were temporarily observed, but the immature nature of circulating granulocytes persisted nine months. Electron microscopic examination and neutrophil function studies revealed no abnormalities. Since previous blood smear evaluation had been normal, it is proposed that the granulocyte anomaly was acquired and may have resulted from an idiosyncratic reaction to one of several chemotherapeutic agents administered.
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A case of a boy is reported with an antibody deficiency syndrome type Bruton leading to a phenocopy of a homozygote Pelger's nuclear anomaly at the age of 10 months. The dominant inheritance and the absence of the anomaly in the peripheral blood of the parents and a sister support the presence of a phenocopy of this leukocyte anomaly. The recurrent bacterial infections are probably the cause of this phenocopy, since the substitution with gammaglobulins did not control the severe antibody deficiency syndrome.
In a series of 306 cases of myeloproliferative disorders followed over a period of 21 years, 18 cases of well-documented acute leukaemia were encountered. Leukaemias were either acute myeloblastic or myelomonocytic and occurred from 6 months to 20 years after the initial diagnosis. Onset was relatively abrupt and the course rapidly fatal with with a median survival of 4 weeks. Due to the prolonged preleukaemic phase, it was possible to carry out a variety of clinical and laboratory observations. While no consistent features were noted, dysplastic haemopoiesis, a fall in leucocyte alkaline phosphatase activity, presence of Pelger-Hüet anomaly and other abnormalities suggest a disturbance in granulocytic maturation. These findings suggest that, following an initial injury to a pleuripotential haemopoietic stem cell, a prolonged 'latent' period occurs and, due to exposure to additional injurious agents or to a lack of cell regulating factors, acute leukaemia develops.
A family has previously been described in which four members with Pelger-Huët (P-H) anomaly suffered from recurrent attacks of abdominal pain and fever, while one member, whose polymorphonuclear leucocytes (PMNs) were also hyposegmented, was asymptomatic. We studied chemotaxis, chemokinesis and spontaneous locomotion of PMNs in the three surviving symptomatic sisters, in their asymptomatic brother and in two asymptomatic members of another family with P-H anomaly. The spontaneous migration of the PMNs of the three sisters was significantly slower both under agarose and in a membrane filter than that of the PMNs of the asymptomatic patients with P-H anomaly. Chemotactic and chemokinetic locomotion of the PMNs of the symptomatic sisters was also slow. Our results suggests that the impaired chemotaxis was due to a defect in the intrinsic locomotor capacity of PMNs rather than in their deformability or their responsiveness to the chemotactic stimulus.
An inbred family of foxhounds with four members expressing the Pelger-Huët (P-H) anomaly is described. The disease-free status of all P-H affected dogs suggests a benign disorder, although review of breeding records indicated a lower percentage of pups weaned (63%) by P-H females compared with the percentage of pups weaned (81%) by outcrossed females throughout the foxhound colony. Light-microscopic examination of blood films from affected dogs revealed 50--67% neutrophils with round, oval, or bean-shaped nuclei and rarely (0.5%) segmented nuclei. Neutrophils examined by electron microscopy showed the nuclei to have a fine nuclear cleft and condensed chromatin and the cytoplasm to have mature heterochromatic granulation. Local P-H neutrophil mobilization through a standard skin abrasion into a chamber containing autologous serum was impaired at all time periods evaluated (1, 4, 8, and 24 hours) compared with the neutrophil mobilization by normal dogs. Antibody response to sheep erythrocyte immunization was also impaired. In vitro reactivity of normal and P-H lymphocytes stimulated by pokeweed mitogen was depressed when lymphocytes were cultured in plasma from a P-H dog but not when cultured in plasma from a normal dog. Vigorous blastogenic responses to phytohemagglutinin by normal and P-H lymphocytes cultured in P-H or normal plasma suggest the presence of a factor(s) in the P-H plasma which interferes with B-lymphocyte reactivity.
Several case reports6-8 in the literature have pointed out that a clinical and morphologic picture resembling that of acute leukemia may be secondary to infection or use of toxic drugs or alcohol. In these cases, the leukemic picture spontaneously returns to normal when the precipitating factor is no longer present. This condition has been termed pseudoleukemia. Unless the history of a precipitating factor is clear, the patient may be wrongly treated for leukemia. In the case reported here, initial findings suggested acute promyelocytic leukemia and chemotherapy was planned, but further investigation indicated that the condition was essentially reactive and related to a presumed viral infection. Even though pseudoleukemia is sometimes described as a leukemoid reaction, we believe that the term "pseudoleukemia" should be reserved for more specific differentiation.
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