ABNORMALITIES of the WHITE BLOOD CELLS Jose R. Villarino, RMT
May 27, 2015
ABNORMALITIES of the WHITE BLOOD CELLS
Jose R. Villarino, RMT
Possible answers: A. NEUTROPHILS B. LYMPHOCYTES C. PATHOLOGIC D. PHYSIOOGIC E. 20-40 % F.
5000-10000/cumm G. 0-3 % H. MALARIA I. AZUROPHILIC
GRANULES
J. ASTHMA K. AUER ROD L. PELGER HUET M. TOXIC
GRANULES N. LEUCOPENIA O. PARASITISM P. SCARLET FEVER Q. DOHLE BODIES R. BASKET CELLS
WBC Normal values: WBC Count = 5,000 – 10,000/cu mm or 5 – 10 x 109/L Differential Count: Neutrophil = 50 – 70 %
Segmenter = 50 – 65 % ; Stab = 0 – 5 %
Eosinophil = 0 – 3 % Basophil = 0 – 1 % Lymphocytes = 20 – 40 % Monocytes = 2 – 6 %
Quantitative abnormalities
Leucocytosis – substantial increase in the WBC count.
- Physiologic increase (no trauma/injury) - Pathologic increase
(trauma/pathology) Leucopenia – substantial decrease in
the WBC count. N.V. = 5,000 – 10,000/cu mm
Differential Count
Neutrophil 50 – 75 %
segmenter 50 – 65 %
stab 0 – 5 %
Eosinophil 0 – 3 %
Basophil 0 – 1 %
Lymphocyte 20 – 40 %
Monocyte 2 – 6 %
The 5 WBC types
Neutrophilia (> 7 – 8 x109/L)
Infections, Inflammation, Metabolic disorders
Acute hemorrhage, corticosteroids Stress, post-surgery, burns, HDN Lithium drugs, neoplasms
Neutropenia (<1.75 – 1.8109/L)
Decreased production - Inherited/acquired stem cell disorder - Benzene toxicity, cytotoxic drugs Increased destruction - Immune mechanism, sequestration BM depression, IM, varicella, Typhoid SLE, hepatitis or any viral infections
Eosinophilia (> 0.7 x 109/L)
Allergic disorders (asthma) Parasitic infections (nematodes) Skin disease (eczema) Hodgkin’s disease Scarlet Fever Pernicious anemia
Eosinopenia (< 0.05 x 109/L)
Stress due to trauma or shock Mental distress Cushing’s syndrome ACTH administration
Basophil (0.3 x 109/L)
BASOPHILIA Chronic myelocyic leukemiaPolycythemia veraHodgkin’s disease
BASOPENIA HyperthyroidismPregnancy
Lymphocytosis (>4.0 x 109/L)
Viral infections ( German measles ) Infectious Mononucleosis (kissing
dis.) Mumps (parotitis), pertussis Tuberculosis, syphilis,
thyrotoxicosis
Lymphopenia
Congestive heart failure, SLE Renal failure Advanced Tuberculosis High levels of adrenal
corticosteroids
Monocytosis (>0.9 x 109/L)
SBE, Syphilis, Tuberculosis Protozoan infections Mycotic or fungal infections Malaria, Systemic lupus
erythematosus Rheumatoid arthritis
Monocytopenia
Lymphocytic leukemia Aplastic anemia
QUALITATIVE CHANGES-WBC
Morphologic abnormalities involving either the nucleus or cytoplasm
Functional abnormalities
Inherited or Acquired Examination of peripheral blood or a
bone marrow evaluation
The White blood cells:
Nucleus details: - Mononuclear or
Polymorphonuclear Granules present: - Granulocytic or Agranulocytic Function: - Phagocytic or Immunocytic
Abnormal granulocyte morphology (acquired)
Toxic granulation, cytoplasmic vacuole
Dohle bodies (Amato bodies) Azurophilic granules Hypersegmentation
Pathological Leucocytes
Abnormal granulocyte morphology
(inherited) Alder-Reilly anomaly - dense
azurophilic granules, mucopolysaccharoidoses
May-Hegglin anomaly - Giant platelets, Dohle-bodies like inclusions seen even in monocytes
Pelger Huet anomaly – failure of normal segmentation of nucleus, bi-lobed nucleus or stab forms only,
“pince-nez nucleus”
Alder Reilly anomaly
May-Hegglin anomaly
Pelger Huet anomaly
Continuation: Chediak Steinbrinck Higashi syndrome –
large lysosomes containing hydrolases and other enzymes. There is anemia,thrombocytopenia, leucopenia and increased susceptibility to infection. There is partial albinism & photophobia.
Also seen in Aleutian mink, mice, cat, cattle & killer whale as caused by abnormal WBCs.
Chediak Higashi syndrome
Other abnormalities: Smudge or basket cells – squash-
degenerated nucleus of WBCs Jordan’s anomaly – fat-containing
vacuoles in WBC cytoplasm, Ichthyosis Twinning deformity Auer rod – rod-like structure seen in
the cytoplasm of myeloblasts, diagnostic for Acute myeloblastic leukemia (AML)
Variants of the Lymphocytes
Plasmacytoid lymphocyte or Turk’s irritation cell
Downey cell (atypical lymphocyte) Transformed lymphocyte (reticular
or pyroninophilic cell) Reider cell – “clover-leaf like
nucleus” Plasma cells
Reactive lymphocytes
Downey cell
Hallmark cell seen in cases of Infectious mononucleosis (kissing disease)
Atypical lymphocyte (stress lymphocyte)
“ballerina skirt cell”
Infectious Mononucleosis
Plasma cells
Ovoid or fibrillary shaped Eccentric location of nucleus Perinuclear halo “cart-wheel pattern or spoke of the
wheel pattern of nucleus” basophilic cytoplasm
Comparative morphology of plasma cells, lymphocytes and NRBC
Inherited abnormalities involving Monocyte-macrophage group
MUCOPOLYSACCHAROIDOSES - Hunter syndrome, Hurler’s disease LIPIDOSES – lipid accumulation - Gaucher’s disease – accumulation of
glucocerebroside due to lack of beta-glucosidase enzyme
- Neimann Pick disease – sphingomyelin and cholesterol accumulation due to lack of the enzyme sphingomyelinase
Monocyte-macrophage abnormality
WBC functions Neutrophil – phagocytic Eosinophil – phagocytic and
damage to larval stages of parasite. Basophil – storage of histamine,
involved in immediate hypersensitivity reaction.
Monocyte – phagocytic, cellular and humoral immunity
Functions….
Lymphocytes – immune leucocytes a)humoral b) lymphokines c)
cytotoxic
- Not obligate end cells - Heterogenous group of cells - Destined to migrate
PHAGOCYTOSIS process
Motility – random movement and directed movement
Recognition Ingestion Degranulation or release of
granules Microbial killing
Inherited functional abnormalities
Job’s syndrome – defective directed movement, characteristic “cold boils”
Lazy leucocyte syndrome – both random & directed movements are defective
Chediak Higashi syndrome – failure to release the granules
Non-neoplastic (non-clonal) disorders of the WBC
Include a) growth regulation abnormalities, b) leukemoid reaction (an increased proliferative response to various stimuli) including bone marrow aplasia and hypoplasia and c) qualitative leucocyte disorders (both acquired & inherited) characterized by deficiency of leucocyte function.
Non-clonal disorders of WBC
Function disordersDefective chemotaxis, phagocytosis, defective killing, CGD, myeloperoxidase deficiency
Quantitative disorders Neutropenia, agranulocytosis,
Leukemoid reaction, Infectious mono
Clonal (neoplastic) disorders of WBC
Derived from a single precursor cell with all the affected cells (progeny) showing features of deviation from the precursor cell.
Myeloproliferative disorders Lymphoproliferative disorders Immunoproliferative disorders
Leukemoid reaction
High WBC count = <50000/cu mm Toxic granulation & Dohle bodies Predominant band forms LAP score = >100 Negative for Philadelphia chromosome - Translocation of genetic material from
long arm of Chromosome 22 to Ch 9
Hodgkin’s disease Belongs to a group of malignant
disorders referred as Lymphomas Lymphomas involved abnormal lymph
node enlargement with replacement or alteration in its histologic characteristic
The neoplastic cell involved is known as the Reed-Sternberg cell. Mostly appears as binucleated with the 2 halves of the cell appearing as mirror images.
Hematopoietic malignancy
Defined as growth or proliferation of one or more clones of abnormal cells. These cells don’t respond to normal control and even produce substances inhibiting growth of normal cells.
These malignancy may be manifested in the peripheral blood as in cases of anemia and thrombocytopenia.
Leukemias In the case of WBC, these malignant
cells may or may not circulate in the peripheral blood. Hence, WBC count may be increased or otherwise.
Should these abnormal cells be present both in the bone marrow and the peripheral blood, the term leukemia is used.
Aleukemic leukemia – if only confined to the marrow and do not circulate.
Classification of the leukemias According to the stem cell line involved - Myeloid – involves the granulocytes,
monocytes, RBCs and megakaryocytes. Also known as myeloproliferative disorders or nonlymphocytic leukemias.
- Lymphoid – involving the B or T cells and may be a leukemia or lymphoma
Classification of leukemias
According to duration (life span)- Acute – days to weeks (3 months) - greater than 30 % blasts forms- Chronic – more than a year (1-2
years) - less than 10 % blast forms
Examples :
Acute myeloid leukemia myeloblast
Chronic myelogenousleukemia
Myelocyte, metamyelocyte & neutro
Acute lymphoblasticleukemia
lymphoblasts
Chronic lymphocyticleukemia
Small mature lymph
ErythroleukemiaDi Guglielmo syndrome
> 50% of the nucleated cells are erythroblasts
Comments on the leukemias: AML – most common form of acute
leukemias in first few months of life, in middle aged group and later years
CML – more common in young & elders ALL – seen among children 2 – 10 y.o. CLL – common among > 60 years old