WBC Disorders

WBC Disorders (Doc Ancheta)
1st Part
Gaucher’s Disease
• Refers to a cluster of AUTOSOMAL RECESSIVE disorders resulting from mutations in the gene
encoding GLUCOCEREBROSIDASE.
• It is the most common LYSOSOMAL STORAGE DISORDER.
• The affected gene encodes GLUCOCEREBROSIDASE, an enzyme that normally cleaves GLUCOSE
residue from CERAMIDE.
• GLUCOCEREBROSIDES accumulate in massive amounts within phagocytic cells throughout the
body in all forms of Gaucher disease.
• The DISTENDED PHAGOCYTIC CELLS, known as GAUCHER CELLS, are found in the SPLEEN, LIVER,
BONE MARROW, LYMPH NODES, TONSILS, THYMUS, and PEYERS PATCHES.
• Gaucher cells are ENLARGED cells, have ONE or MORE DARK ENCENTRICALLY PLACED NUCLEI, and
FIBRILLARY TYPE OF CYTOPLASM likened to CRUMPLED TISSUE PAPER
Periarteriolar Lymphoid
Sheath (PALS)
• Splenic red pulp has numerous clusters Gauchers cells
Sinus Histiocytosis
• Aka RETICULAR/SINUS HYPERPLASIA. Refers to an INCREASE in the NUMBER and SIZE of the cells
that line LYMPHATIC SINUSOIDS
• NON-SPECIFIC finding indicative of a REACTIVE PROCESS.
• Prominent in LYMPH NODES draining cancers such as CARCINOMA OF THE BREAST.
• Lymphoid nodules/ follicles @ CORTEX. Drainage happens @ MEDULLA where sinuses,

histiocytes, and blood vessels can be found.
• Germinal center is marked by LIGHT AND DARK ZONES.
• In this slide, medulla exhibits expansion of the sinuses

• Pale areas exhibit hyperplasia of the macrophages (histiocytes). Tissue is @medulla
• Capillaries exhibit hyperplasia and hypertrophy of the endothelial lining. Capillaries

Follicular hyperplasia (REACTIVE/FOLLICULAR HYPERPLASIA)
• is caused by stimuli that activate HUMURAL IMMUNE RESPONSES

• It is defined by the presence of LARGE OBLONG GERMINAL CENTERS (SECONDARY FOLLICES),
which are surrounded by a COLLAR OF SMALL RESTING NAÏVE B CELLS (THE MANTLE ZONE).
FEATURES: (NON NEOPLASTIC)
o PRESERVATION OF THE LYMPH NODE ARCHITECTURE, including the INTERFOLLICULAR T-

CELL ZONES and the SINUSOIDS.
o MARKED VARIATION in the SHAPE AND SIZE of the FOLLICLES.
o The presence of FREQUENT MITOTIC FIGURES, PHAGOCYTIC MARCROPHAGES, and
recognizable LIGHT AND DARK ZONE, all of which tend to be absent from neoplastic
follicles.
TONSIL
• Lined by stratified squamous epithelium. Beneath that is the proliferation of lymphoid follicles.
• There is proliferation of lymphoid follicles all throughout the tissue.
Passive Congestion of the Spleen
• Caused by CHRONIC VENOUS OUTFLOW OBSTRUCTION

• CIRRHOSIS OF THE LIVER is the main cause of MASSIVE CONGESTIVE SPLENOMEGALY.
o “PIPE-STEM” HEPATIC FIBROSIS OF SCHISTOSOMIASIS
o ALCOHOLIC CIRRHOSIS
• Congestive splenomegaly is also caused by OBSTRUCTION OF THE EXTRAHEPATIC PORTAL VEIN
OF SPLENIC VEIN
o SPONTANEOUS PORTAL VEIN THROMBOSIS or INFLAMMATION IF THE PORTAL VEIN
(PYLEPHLEBITIS)
• NORMAL
• Congested spleen where you can appreciate the red pulp is expanded and there is constriction of
the space occupied by white pulp (marked by the central artery). Spleen is HEAVIER.
• Condition associated in the blood. There is HYPERSPLENISM. The spleen is HYPERFUNCTIONING.
There is SEQUESTRATION of enough red blood cells to produce 1. ANEMIA, 2. THERE IS
DEPLETION OF THE THROMBOCYTES, 3. INCREASE VULNERABILITY OF THE SPLEEN TO BLUNT
TRAUMA WHICH PRODUCES BLEEDING.
• The red pulp is solely occupied by red blood cells. PASSIVE CONGESTION OF THE SPLEEN
2nd Part
Plasmacytoma/ Multiple Myeloma
• Is a plasma cell neoplasm commonly associated with lytic bone lesions, hypercalcemia, renal
failure, and acquired immune abnormalities.
• Usually present as DESTRUCTIVE PLASMA CELL TUMOR (PLASMACYTOMAS) involving the axial
skeleton
• The marrow contains an increase number of plasma cells, which usually constitute more than
30% of the cellularity.
• Pathogenesis: Rearrangements involving the IgH (Immunoglobulin Heavy chain) locus and
various proto-oncogenes.
Follicular Lymphoma (Nodular Lymphoma)
• Likely arises from germinal center B cells and is strongly associated with chromosomal
translocations involving BCL2
• Its hallmarks is a (14;18) translocation
• Nodular or nodular and diffuse growth pattern of growth
• Absence of light and dark zones in the germinal center
• Two principal cell types:
o CENTROCYTES- Small cells with irregular or cleaved nuclear contours and scant
cytoplasm.
o CENTROBLASTS- Larger cells with open nuclear chromatin, several nucleoli, and modest
amounts of cytoplasm
Large cell lymphoma
• The most common form of Non Hodgkin lymphoma

• Aka Diffuse large B cell lymphoma (DLBCL)
• The common features are a relative large cell size (usually 4 to 5 times the diameter of a small
lymphocyte) and a diffuse pattern of growth.
• Pathogenesis: Dysregulation of BCL6, a DNA-binding zinc-finger transcriptional repressor that is
required for the formation of normal germinal centers. B CELL LINEAGE
o Translocations that have in common a breakpoint in BCL6 at chromosome 3q27
Hodgkin Lymphoma (Nodular sclerosing type)
• This is the most common form of HL, constituting 65-70% of cases.

• Classical Hodgkin Lymphoma
• Neoplastic cells are REED STERNBERG cells
• RS cells release factors that induce accumulation of reactive lymphocytes, macrophages and
granulocytes
• UNCOMMONLY ASSOCIATED WITH EBV
• The prognosis is EXCELLENT
Gastric Lymphoma
• Nearly 5% of all gastric malignancies are primarily lymphomas, the most common of which are
indolent extranodal marginal zone B cell lymphomas.
• In the gut, these tumors are often referred to as LYMPHOMAS OF MUCOSA-ASSOCIATED
LYMPHOID TISSUE (MALT), or MALTOMAS
• Extranodal marginal zone B-cell lymphomas usually arise at sites of chronic inflammation.
• Commonly transforms into Diffuse Large B Cell Lymphoma.
• H. pylori
Granulocytic Leukemia
• Acute myelogenous leukemia (AML) that invaded extramedullary organs and tissue
• Comprised of immature large myeloblastic cells that has high N/C ratio, scanty cytoplasm and
prominent nucleoli.

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WBC Disorders (Doc Ancheta)

• Lymphoid nodules/ follicles @ CORTEX. Drainage happens @ MEDULLA where sinuses,

• In this slide, medulla exhibits expansion of the sinuses

• Capillaries exhibit hyperplasia and hypertrophy of the endothelial lining. Capillaries

• is caused by stimuli that activate HUMURAL IMMUNE RESPONSES

FEATURES: (NON NEOPLASTIC)

o PRESERVATION OF THE LYMPH NODE ARCHITECTURE, including the INTERFOLLICULAR T-

• Caused by CHRONIC VENOUS OUTFLOW OBSTRUCTION

Plasmacytoma/ Multiple Myeloma

• The most common form of Non Hodgkin lymphoma

• This is the most common form of HL, constituting 65-70% of cases.

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