re: immunodeficiency

From: Nebe, Thomas C. (thomas.nebe@ikc.ma.uni-heidelberg.de)
Date: Mon Jan 18 1999 - 15:36:57 EST


> Dear collegue,
> the case you presented seems to show an increase in neutrophils.  The
> abslolute lymph count seems to be 2250 per µl.  60% CD19 gives ca. 1300 B
> cells per µl that is above normal. T cells seem to be at the lower limit
> or less.
> Immunoglobulin levels in gram per liter are above normal, but you didn“t
> gave units.
> What is the clinical presentation? Fever, lymph nodes, spleen, time
> course, weight loss? Bacterial infections may also direct towards
> neutrophil disorders (if immunoglobulins are high).
> What is the morphology (smear)?  Carefully review a complete slide.
> In our CVIDs we had no increase in B cells.  So lymphoma needs to be
> excluded even the probability is not high.
> The kappa lambda (if its polyclonal) may be negative on some immature B
> cells, that“s normal.
> The rest of B markers, if expressed at normal densities, are useful for
> NHL but do not help for CVID.
> IgM, IgD and IgG on CD19/20 and T cell activation tests (skin and LTT) may
> help for CVID to show maturational disturbances.  IgG subclasses help
> further to testify CVID, where immunoglobulins are the strongest
> arguments.
> Because of the low frequency it is also beyond our lab tests to look for
> defects in receptor chains (eg. Il-2 etc). The CVIDs are diverse and
> require broad panels of molecular genetic tests.
> It may be worthwile from a clinical point of view to look for chronic
> viral infections as they give similar pictures (igs being mostly elevated)
> or might persist and worsen pictures of CVID.  The CD4/8 ratio argues
> against several ones but not against hepatitis and others.
> Is HLA-DR on B cells normal (bare lymphocyte syndrome)?  Unlike stated in
> textbooks you find inborn IDs even in young adults.  Eg. we just had a
> Chediak Higashi Syndrome in a 27 year old women.
> I hope this helped a little bit to solve your problem.
> Regards
> Thomas Nebe
> 
> Dr.med. C. Thomas Nebe
> Universitaetsklinikum Mannheim
> Institut für Klinische Chemie
> Theodor-Kutzer-Ufer 1-3
> D-68167 Mannheim
> Tel.  +49 621 383-3485
> FAX  +49 621 383-3819
> thomas.nebe@ikc.ma.uni-heidelberg.de
> PSE 75-1445
> 



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