Autoimmune Cytopenias in Chronic Lymphocytic Leukemia

Table 2

Recommendations for the diagnosis of CLL-associated autoimmune cytopenias.

Autoimmune Hemolytic Anemia
(1) Positive DAT*
(2) Reticulocytosis
(3) Elevated serum LDH
(4) Elevated serum indirect bilirubin*
(5) Reduce serum haptoglobin*
(6) Erythroid hyperplasia in bone marrow
Autoimmune Pure Red Cell Aplasia
(7) Severe normocromic-normocytic anemia*
(8) Reticulocytopenia*
(9) Erythroid precursors ≤1% of bone marrow cells*
(10) No parvovirus B19 infection by polymerase chain reaction assay*
(11) DAT negativity*
(12) No presence of hemolysis (normal haptoglobin, unconjugated bilirubin, LDH)*
(13) More than 4–8 weeks from the last chemotherapy infusion*
Immune Thrombocytopenia
(1) Rapid and “unexplained” fall in the platelet count*
(2) Augmented number of megakaryocytes in the bone marrow*
(3) More than 4–8 weeks from the last chemotherapy infusion*
Autoimmune granulocytopenia
(1) Persistent and “unexplained” neutropenia*
(2) Decreased or absent granulocyte precursors in bone marrow*
(3) Presence of anti-neutrophilantibodies
(4) More than 4–8 weeks from the last chemotherapy infusion*

DAT: direct antiglobulin test.
LDH: lactatedeydrogenase serum levels.
Note that some of these criteria can be not always applicable for patients with CLL, in particular in the case of AIHA (i.e., absence of recticulocytosis due to bone marrow infiltration and/or elevated LDH without AIHA in case of aggressive CLL; ITP is not always rapid). Furthermore, DAT may be negative in patients with AIHA complicating-CLL.
*Marks criteria that, in our opinion, should be considered more relevant for AC diagnosis in patients with CLL.