WBC disorders Flashcards
four instances to worry about dysfunction
1 - Recurrent bacterial infections
2 - Infections of unusual sites
3 - Infections with unusual pathogens
4 - Chronic gingivitis or aphthous ulcers
name four congenital disorders of neutrophil function
1 - leukocyte adhesion deficiency
2 - hyperimmunoglobulin E syndrome
3 - Chediak-Higashi syndrome
4 - chronic granulomatous disease
mechanism, signs, and treatment of leukocyte adhesion deficiency
- adhesion and rolling defect
- recurrent bacterial infections, neutrophilia but no pus
- treatment is stem cell transplantation
mechanism, signs, treatment of hyperimmunoglobulin E syndrome
- chemotaxis defect
- Chronic dermatitis, recurrent staph, candidal and lung infections
- High IgE levels
- Tx is generally supportive with prophylactic antibiotics
causes of acquired disorders of neutrophil function
- myelodysplasia/myelodysplastic syndrome (MDS)
- alcoholism
- metabolic disorders
relevance of low eosinophil count
- none, not clinically significant
most common cause of eosinophilia in world and in developed countries
- world - parasitic infections
- developed countries - atopic allergic diseases
criteria for significant and severe eosinophilia
significant - absolute count greater than 1500 cells/ul for 6 weeks
severe - greater than 5000 cells/ul
primary and secondary causes of eosinophilia
primary (neoplastic/malignant) - hematological malignancies with evidence of clonal expansion secondary (reactive) - Parasitic infections - Allergies, Asthma - Meds (can be virtually any drug) & toxins - Autoimmune disorders - Lymphomas - Chronic myelogenous leukemia (CML)
subsets of primary eosinophilia
- acute eosinophilic leukemia (rare form of AML)
- chronic eosinophilic leukemia (Some have the FIP1L1/PDGFRA mutation that responds to tyrosine kinase inhibitor treatment with imatinib (Gleevec))
NAACP for eosinophilia
Neoplasm Allergy Asthma Collagen vascular disease Parasitic infection
important to the workup for eosinophilia is:
complete history and physical
important diagnostic studies for eosinophilia
- CBC and differential
- Serial stools for Ova & Parasites
- Connective tissue serologic studies such as ANA
- Bone marrow or tissue biopsy
relevance of low basophil levels
none, not clinically relevant
when is a high basophil level often seen?
with myeloproliferative disorders, particularly chronic myelogenous leukemia
causes of monocytosis
- chronic infections
- bone marrow recovery after chemo
- autoimmune and CT disorders
- granulomatous disease
- primary malignancies (acute myelomonocytic leukemia or chronic monocytic leukemia)
- secondary malignancies (Hodgkin’s lymphoma)
causes of monocytopenia
severe infections bone marrow failure - MDS - aplastic anemia hairy cell leukemia (usually with concurrent neutropenia)
lab criteria for lymphocytosis
ALC > 4000 cells/ul
causes of non-malignant lymphocytosis
acute infection - EBV (mono)
chronic infection - brucellosis, TB
drug - hypersensitivity reaction
causes of malignant lymphocytosis
- B cell chronic lymphocytic leukemia (CLL)
- other chronic leukemias (PLL, hairy cell, plasma cell)
- lymphomas
- acute lymphoblastic leukemias
in B cell cytometry studies what is a normal kappa:lambda ratio?
70:30
what does a strongly imbalanced kappa:lambda ratio tell us?
clonality
how do we test of clonality in T cells and why?
PCR or Western Blot of T cell receptor because T cells do not have good surface markers like B cells
define lymphocytopenia
absolute lymphocyte count under 1000 cells/uL
