Table VII.
Warning signs of PIDD for hematologists
| Clinical occurrences | PIDD | Laboratory tests |
|---|---|---|
| Thrombocytopenia with small-sized platelets | Wiskott-Aldrich syndrome (WAS) | CBC including platelet number and size (small sized platelets); CMI, AMI |
| Other symptoms: eczema and recurrent infections | ||
| X-linked thrombocytopenia | ||
| Autoimmune cytopenias (autoimmune anemia, thrombocytopenia and neutropenia) | Common variable immunodeficiency | AMI, ANA |
| Other features: recurrent infections | ||
| Fever, splenomegaly without evidence of malignancy, cytopenias | Hemophagocytic lymphohistiocytosis (HLH) | CBC, triglycerides, ferritin, EBNA |
| Lymphadenopathy + Splenomegaly | Autoimmune lymphoproliferative disease | Increased number of alpha beta double-negative T cells (CD3 + CD4-CD8-), ANA, RCP |
| Excluding neoplasias and infections | Apoptosis defects | |
| Quantitative and qualitative defects of neutrophils (neutropenia and neutrophilia) | Neutropenias | P |
| Chronic granulomatous disease (CGD) | ||
| Leukocyte adhesion deficiency | Leukocytosis, CD18+ cells | |
| Partial albinism, Chediak-Higashi or Griscelli syndrome | Enlarged cytoplasm granules |
Abbreviations: AMI antibody mediated immunity, ANA antinuclear antibodies, CBC complete blood count, CMI cellular mediated immunity, EBNA Epstein-Barr nuclear antigen, P phagocytosis, RCP reactive C protein