Introduction

For a considerable time the finding of weak Kell antigens in the dental student Hugh McLeod had remained an oddity only known to immunohematologists, such as the groups of F.H. Allen in Boston and of W.L. Marsh in New York [1]. The occurrence of his peculiar "McLeod" erythrocyte phenotype in boys with chronic granulomatous disease (CGD) was not noted until after a decade had passed [21, 34] and neither was the shape-change of the antigen-deficient red cells because of acanthocytic deformation [19, 49].

The McLeod syndrome (MLS) with X-linkage and a variety of features apart from red cell involvement was delineated by W.L. Marsh only in the 1970s. On a lecture trip through New Zealand, local physicians asked him to consult concerning a family with "acanthocytosis associated with haemolytic anaemia" (Fig. 1) . After his return, Marsh on November 25, 1975 wrote a letter to Dr. McLeod who was in regular contact with the New York Blood Center as a blood donor for CGD patients: "I thought you would like to know that you are no longer unique for we have now tested a large family sent from New Zealand and two of the male

Fig. 1 Pedigree of the "S" family with McLeod syndrome due to an XK deletion [33, 45]. The index patient of the present communication, case WA, is marked by an arrow and corresponds to patient III-7 of [33] (as well as to II-4 of [45] and case 8 of [13]). Cases A, B, and C in [33] correspond to III-2 (also case 7 of [13]), III-17 (also case 2 of [6] and case 5 of [13]), and III-16 (also case 1 of [6] and case 6 of [13]), respectively. Please note that the two brothers B and C had erroneously been described as cousins or single case ("S/H") in the past [6, 24, 26]

Fig. 1 Pedigree of the "S" family with McLeod syndrome due to an XK deletion [33, 45]. The index patient of the present communication, case WA, is marked by an arrow and corresponds to patient III-7 of [33] (as well as to II-4 of [45] and case 8 of [13]). Cases A, B, and C in [33] correspond to III-2 (also case 7 of [13]), III-17 (also case 2 of [6] and case 5 of [13]), and III-16 (also case 1 of [6] and case 6 of [13]), respectively. Please note that the two brothers B and C had erroneously been described as cousins or single case ("S/H") in the past [6, 24, 26]

members have the red cell McLeod phenotype. ... We have been able to show that some female members in the New Zealand family are carriers of the gene."

The New Zealand "S" family [45] was studied repeatedly for the multi-system features that were first properly noted in its members [32, 33, 42] and was also important in the first attempts to define the syndrome's molecular basis [6, 24, 26]. The first autopsy of a male (WA) with MLS is from this family, briefly mentioned in reviews of MLS [13, 33, 45] and individually reported with little detail in a book chapter [8] and an abstract [9]. Here we provide the first comprehensive account of the clinical and pathological findings of this patient.

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