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ABNORMALITIES of the WHITE BLOODCELLS Jose R. Villarino, RMT
Possible answers: A. NEUTROPHILS J. ASTHMA B. LYMPHOCYTES K. AUER ROD C. PATHOLOGIC L. PELGER HUET D. PHYSIOOGIC M. TOXIC E. 20-40 % GRANULES F. 5000-10000/cumm N. LEUCOPENIA G. 0-3 % O. PARASITISM H. MALARIA P. SCARLET FEVER I. AZUROPHILIC Q. DOHLE BODIES GRANULES R. BASKET CELLS
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 (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”
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.
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
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 plasmacells, lymphocytes and NRBC
Inherited abnormalities involvingMonocyte-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
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.
Clonal (neoplastic) disordersof 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 myeloblastChronic myelogenous Myelocyte, metamyelocyte &leukemia neutroAcute lymphoblastic lymphoblastsleukemiaChronic lymphocytic Small mature lymphleukemiaErythroleukemia > 50% of the nucleated cellsDi Guglielmo syndrome 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