Robert C. Neerhout

Combined immunodeficiency presenting as the Letterer-Siwe syndrome

Four infants with skin rash, hepatosplenomegaly, lymphocytosis, eosinophilis, and histiocytic infiltration of the lymph nodes and skin are described; in each of these infants an initial diagnosis of the Letterer-Siwe syndrome was made. Postmortem findings of thymic dysplasia and poorly differentiated, lymphopenic peripheral lympoid tissue in each of the four infants, as well as antimortem clinical findings in one, established a diagnosis of severe combined immunodeficiency. From these and similar cases in the literature, we postulate that the Letterer-Siwe syndrome may not be an unusual presentation of combined immunodeficiency. Appropriate immunologic studies will help to differentiate the two disorders.

Immunologic Reconstitution in Severe Combined Immunodeficiency without Bone-Marrow Chromosomal Chimerism

Abstract An 11-month-old male infant with severe combined immunodeficiency was immunologically reconstituted by bone-marrow transplantation from a female sibling whose cells were HL-A compatible on...

Late death fromneuroblastoma

7. Holland, N. H., Jurichs, R., and Clemons, G.: Detection of asymptomatic urinary tract infection in girls, J. Ky. Med, Assoc. 67: 662, 1969. 8. Dodge, W. F., West, E. F., Fras, P. A., and Travis, L. B.: Detection of bacteriuria in children, J. PEDIATR. 74: 107, 1969. 9. Wahen, M. G., Jr., and Kunin, C. M.: Significance of borderline counts in screening programs for bacteriuria, J. PEmATm 78: 246, 1971. 10. Dodge, W. F., West, E. F., Holloway, J. G., Bridgforth, E. B., and Travis, L. B.: Pattern of maternal desires for child health care, Am. J. Public Health 60: 1421, 1970.

Reversibility of the erythrocyte lipid abnormalities in hepatic disease

The alterations in the lipid composition of the erythrocyte membrane in patients with hepatic disease are reversible as hepatic function improves. Improvement in the lipid pattern appears to follow more closely the improvement of liver function than the changes in the plasma lipids in the 5 patients described. Morphologic alterations of erythrocytes with changes in the membrane lipids are noted; these too are reversible as liver function improves.

Hemolytic Anemia in Infectious Mononudeosis

M.D., ~epax’ase&t!~; ’o~:. ~ Pediatrics, UCLA School of i edidne Los Angeles, ~eaN..WO’S4. ~.~ ’.~&dquo; ~’~:~ ~ ~ ~&dquo;’ ~ ~ ~f’ ~ ~ .~~~~ ~ . A ACUTE hemolytic anemia and thrombocytopenia are two uncommon complications of infectious mononucleosis ~I~~~: The rarity of acute hemolytic anemia with I1~I is emphasized by finding that this complication was not noted in five large series comprising 1,113 patients.1,5,9, 13, 14 Although standard hematology texts and review articles point out this complication, a recent review

Experience with incompatible maternal donors for bone marrow transplantation

SummaryMarrow transplantation in aplastic anemia and leukemia has generally been limited to siblings who have been histocompatible at both the serological (A and B) and lymphocyte determined (D or MLC) loci of the HLA system. We studied three male patients, two with aplastic anemia and one with acute myelogenous leukemia, who received transplants from their histoincompatible mothers. MLC studies between donors and recipients showed varying degrees of stimulation. Definite engraftment occurred in one patient and transient engraftment in another. Engraftment in the third patient could not be evaluated. In the patient with sustained engraftment, there was clinical evidence of severe graft versus host disease (GVHD) however, this was not substantiated by histologic findings. This preliminary study suggests that MLC incompatibility may be more of an indicator of the risk of GVHD than of bone marrow rejection. If more effective control of GVHD can be accomplished, marrow transplantation between MLC-reactive individuals may become feasible.ZusammenfassungKnochenmarktransplantationen bei aplastischer AnÄmie und akuter LeukÄmie wurden bisher fast ausschlie\lich bei Patienten durchgeführt, für die ein Geschwister-spender zur Verfügung stand, der unter Berücksichtigung sowohl der serologischen (A und B) als auch der zellbedingten (D) Loci des HLA-Systems als histokompatibel angesehen werden konnte. In der vorliegenden Arbeit wird über zwei Patienten mit aplastischer AnÄmie und einen Patienten mit akuter myeloischer LeukÄmie berichtet, die Knochenmarktransplantate von ihren nicht histokompatiblen Müttern erhielten. Die gemischten Lymphozytenkulturen zeigten Stimulation wechselnder StÄrke zwischen Spender und EmpfÄnger. Bei einem Patienten kam es zu einem stabilen, bei einem weiteren zu einem vorübergehenden Anwachsen des Transplantats; bei einem dritten war eine genaue Beurteilung nicht möglich. Bei dem Patienten mit stabilem Transplantat wurden klinische Anzeichen einer Spender-gegen-Wirt-Reaktion fest-gestellt, die allerdings histologisch nicht bewiesen werden konnten. Diese vorlÄufigen Ergebnisse lassen uns vermuten, da\ MLC-InkompatibilitÄt mehr ein Indikator einer Spender-gegen-Wirt-Reaktion, als der einer Transplantat-Absto\ung ist. Es erscheint nicht ausgeschlossen, da\ die Entwicklung von Methoden zur erfolgreichen Behand-lung der Spender-gegen-Wirt-Reaktion Transplantationen von MLC-positiven Spendern ermöglicht.

Review : Disorders of the Red Cell Membrane A review of biochemical and physiologic altera tions of erythrocyte membranes which may lead to morphologic changes and shortened cell survival

Morphologically similar cells can be seen in such varied conditions as liver disease, renal disease, a-betalipoproteinemia, malab sorption states, congenital nonspherocytic anemias, thalassemia, iron deficiency, pykno cytosis, hemolytic-uremic syndrome, erythro cyte enzyme deficiencies, etc. The common denominator in these condi tions may be an increase in membrane rigid ity, resulting from a diversity of causes. These would include altered protein-lipid- carbohydrate composition, abnormally struc tured hemoglobins, the presence of Heinz bodies due to altered glycolysis or unstable hemoglobins. The end result of such altered membrane elasticity, regardless of cause, would be fragmentation and destruction of the red cells during passage through small vascular channels, particularly in the spleen. Changes in erythrocyte stromal composi tion often seem to reflect more generalized systemic metabolic disorders. Whether any of the alterations of membrane composition are causally related to either the morpho logic changes or the shortened cell survival still remains to be proven.Morphologically similar cells can be seen in such varied conditions as liver disease, renal disease, a-betalipoproteinemia, malab sorption states, congenital nonspherocytic anemias, thalassemia, iron deficiency, pykno cytosis, hemolytic-uremic syndrome, erythro cyte enzyme deficiencies, etc.The common denominator in these condi tions may be an increase in membrane rigid ity, resulting from a diversity of causes. These would include altered protein-lipid- carbohydrate composition, abnormally struc tured hemoglobins, the presence of Heinz bodies due to altered glycolysis or unstable hemoglobins. The end result of such altered membrane elasticity, regardless of cause, would be fragmentation and destruction of the red cells during passage through small vascular channels, particularly in the spleen. Changes in erythrocyte stromal composi tion often seem to reflect more generalized systemic metabolic disorders. Whether any of the alterations of membrane composition are causally related to either the morpho logi...